News & Events
3. A 46-year-old potential kidney donor came forward to donate to his brother. 111 mismatch, no DSA. CT showed angiomyolipoma in the left kidney (4.6 cm in diameter).
- Will you accept this donor?
- What is angiomyolipoma?
- What are the risks involved?
- Is this image adequate?
- What are the other diseases associated with angiomyolipoma?
178 Comments
Leave a Reply
You must be logged in to post a comment.
Copyright © 2024 World Kidney Academy. All Rights Reserved. ICOM
You cannot copy content of this page
Yes, I will accept the donor
As unilateral small angiomyolipoma is not contraindicated for donation.I will take this kidney and do ex vivo excision and save normal one to the donor
Angiomyolipoma
Is the most common kind of benign renal tumor , affecting about 0.2% to 0.6% of people . It is made of blood vessels, muscle cells and fat .
It can cause retroperitoneal bleeding. That may be life-threatening
Is this image adequate
MRI is the best modality for diagnosis
Other diseases associated
Lymphangioleiomyomatosis
Neurofibromatosis type 1
Tuberous sclerosis complex
1-
Yes I will accept this patient after well evaluate his condition. Especially if it was an isolated AML.
A literature search was conducted utilizing MEDLINE, EMBASE, and Cochrane databases from inception through May 15, 2018 (updated on October 2019).
The conclusion of this study was reassuring for the safety of donors with AML (without TSC or LAM) as well as their recipients without evidence of malignant transformation of AML. As such, this can also positively impact the donor pool by increasing the number of available kidneys
2-
Renal angiomyolipoma (AML) is the most frequent mesenchymal tumor of the kidney.
Renal AML is a heterogeneous, triphasic tumor with varying elements of smooth muscle, adipose tissue, and vascular elements.
AML can occur sporadically with an incidence ranging from 55% to 80% or in association with the tuberous sclerosis complex (TSC) in about 20%–30% and very rarely as sporadic lymphangioleiomyomatosis (LAM)
3-
Risk f for malignant AML include
size >7 cm, tumor necrosis, and epithelioid carcinoma-like pattern.
4-
MRI abdomen is very sensitive in diagnosing and distinguishing fat-poor AML lesions from renal malignancies.
5-
TS
LAM
Reference
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8052899/
Desiree Garcia Anton.Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic reviewUrol Ann. 2021 Jan-Mar; 13(1): 67–72.
Angiomyolipoma is a benign tumor that consists of adipose tissue , blood vessels and smooth ms cells .
It Carries the risk of malignant transformation
it is usually accidentally discovered.
can be bilateral in both kidneys or unilateral
If the donor has AML split kidney function by isotope scan should be done to assess kidney function.
Usually the best kidney should be left to the donor
and better for living kidney donation if excision of AML exvivo is easy so should be removed before transplant
Renal angiomyolipoma (AML) is the most frequent mesenchymal tumor of the kidney. Although there is a rare possibility of malignant transformation of AML, this risk has not been studied in immunosuppressed patients. The safety of donors with AML and their kidney transplant recipients has not been well established.
These findings are reassuring for the safety of donors with AML (without TSC or LAM) as well as their recipients without evidence of malignant transformation of AML. As such, this can also positively impact the donor pool by increasing the number of available kidneys.
https://pubmed.ncbi.nlm.nih.gov/33897168/
This donor can be accepted in this case after performing DMSA scan and assessing the split function of the both kidneys. After evaluation of the mass being of small size and unilateral, this kidney if displays the less eGFR functional activity, ex-vivo excision of the mass perioperatively can be proceeded.
Angiomyolipoma is believed to a benign vascular lesion, more prevalent in females, can be accidently discovered being asymptomatic in most conditions.
Risks are mainly hemorrhage after rupture due to hypertension or anticoagulation therapy. Malignant transformation is also a serious condition.
Abdominopelvic MRI is the best imaging modality in this case as it offers perfect soft tissue differentiation as well as proper localization of the mass and excludes lymph nodes involvement if malignant transformation has occurred.
Other lesions could be associated are primarily Tuberous sclerosis complex and Lymphangioleiomyomatosis.
· Will you accept this donor?
· What is angiomyolipoma?
· Angiomyolipomas (AMLs) are benign or less commonly malignant neoplasms occurring in the kidney.
· More common in females
· Can be accidentally discovered in asymptomatic case at a rate of 0.1 up to 2.2%
· They are composed of adipose tissues, varying amounts of tissues resembling blood vessels and smooth muscle.
· It could be sporadic or associated with Tuberus sclerosis
· What are the risks involved?
Is this image adequate?
· We need to do a CT or MRI to assess the other kidney.
· MRI abdomen is the imaging of choice. It can differentiate fat-poor AML from renal malignancies. Renal AML can be classified as fat-rich, fat-poor and fat-invisible. The fat-poor and fat-invisible can rapidly grow and undergo malignant transformation.
What are the other diseases associated with angiomyolipoma?
· Tuberous sclerosis complex
· Lymphangioleiomyomatosis
References:
1.Rofaiel G, Pan G, Campsen J, Kim R, Hamilton B. Successful Utilization of a Live Donor Kidney with Angiomyolipoma. Cureus. 2020;12(2).
2. Anton DG, Kovvuru K, Kanduri SR, Aeddula NR, Bathini T, Thongprayoon C, et al. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urology annals. 2021 Jan-Mar;13(1):67-72.
3. Gopalakrishnan N, Dhanapriya J, Sakthirajan R, Dineshkumar T, Balasubramaniyan T, Haris M. Angiomyolipoma of donor kidney: Successful transplantation and 5-year follow-up. Indian journal of nephrology. 2016 Mar-Apr;26(2):131-3.
4. Andrews PA et al; British Transplantation Society/Renal Association UK guidelines for living donor kidney transplantation 2018;summary of updated guidelines of transplantation 2018;July;102(7)e307.
Will you accept this donor?
Yes, i will accept this donor. Angiomyolipoma in a donor is not a contraindication for donation,irrespective of size.
What is angiomyolipoma?
Angiomyolipomas (AMLs) are benign neoplasms occurring in the kidney and are composed of varying amounts of tissue resembling blood vessels, smooth muscles, and adipose tissue.
What are the risks involved?
Those with greater than 4cm of angiolipoma have high risk of bleeding including retroperitoneal bledding.
Classic, “fat-rich” AMLs (determined by imaging) do not undergo malignant transformation and are therefore low risk. By contrast, “fat-poor” or “fat-invisible” AMLs may contain epithelioid cells and sometimes undergo malignant transformation. Such lesions should be biopsied. Features consistent with a high risk for malignant transformation include ≥70% epithelioid cells, vascular invasion, ≥2 mitotic figures per 10 high-power fields, atypical mitotic figures, and necrosis.
Is this image adequate?
No MRI and Angiography may be needed to assess look for fat and vascularity.
Renal AMLs are often associated with:
A 46-year-potential kidney donor to his brother.
111 mismatch, no DSA.
CT showed angiomyolipoma in the left kidney (4.6 cm)
● Will you accept this donor?
☆ If the donor with AML doesn’t have TSC or LAM I will accept him (ECD).
☆ Also I will need a team including urologist, general surgeon, nephrologist, and radiologist for helping navigate the donor with AML .
☆ I can do ex vivo excision of the AML .
☆ Close surveillance and monitoring is recommended. Besides physical examination and imaging studies at 6 months and annually
● What is angiomyolipoma?
☆ Renal angiomyolipoma (AML) is the most frequent mesenchymal tumor of the kidney.
☆ It is a heterogeneous, triphasic tumor with varying elements of smooth muscle, adipose tissue, and vascular elements
☆ It persist as a typically small, solitary, unilateral, and predominantly seen middle-aged women.
● What are the risks involved?
Although there is a rare possibility of malignant transformation of the AML, the risk is not studied in immunosuppressed patients such as transplant recipients.
● Is this image adequate?
☆ We need MRI abdomen which is sensitive in diagnosing and distinguishing fat-poor AML lesions from renal malignancies.
☆ Renal biopsy can provide an accurate diagnosis of AML, but it is rarely performed due to the risk of tumor rupture and hemorrhage.
● What are the other diseases associated with angiomyolipoma?
☆ Tuberous sclerosis complex (TSC) in about 20%–30%
☆ In rare cases sporadic lymphangioleiomyomatosis (LAM).
Desiree Garcia Anton, Karthik Kovvuru, Swetha R Kanduri, Narothama Reddy Aeddula,1 Tarun Bathini,2 Charat Thongprayoon,3 Wisit Kaewput,4 Karn Wijarnpreecha,5 Kanramon Watthanasuntorn,6 Sohail Abdul Salim, Praise Matemavi,7 Pradeep Vaitla, Franco Cabeza Rivera, and Wisit Cheungpasitporn.Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review.Urol Ann. 2021 Jan-Mar; 13(1): 67–72.
Will you accept this donor?
I will accept him
There has been growing literature with case reports of successful renal transplantation after excision of AML.
Postoperative 5-year follow-up did not demonstrate a significant increase in size of the tumor despite immunosuppression.
Although it might not solve the global problem of kidney transplant shortage, it could certainly pave the way for the recipients whose only available donors have benign renal AML.
patients with small, sporadic, unilateral asymptomatic renal AML can be accepted with favorable donor and recipient outcomes.
What is angiomyolipoma?
Renal angiomyolipoma (AML) is the most frequent mesenchymal tumor of the kidney.
AML are benign renal tumors
Renal AML is a heterogeneous, triphasic tumor with varying elements of smooth muscle, adipose tissue, and vascular elements.
Renal AML can occur sporadically with an incidence ranging from 55% to 80% or in association with the tuberous sclerosis complex (TSC) in about 20%–30% and very rarely as sporadic lymphangioleiomyomatosis (LAM).
The sporadic forms of AML are typically small, solitary, unilateral, and predominantly seen middle-aged women.
The diagnosis of the AML is commonly asymptomatic and could be detected incidentally on imaging in more than 80% of the cases.
Non renal AML can be found in lymph nodes, renal vein and retroperitoneum
What are the risks involved?
in tumors >4 cm in size, they could present with clinical manifestations of left flank pain, tender mass, hematuria, and fatigue.
Less than 15% manifests as hemorrhage at the presentation (Wunderlich syndrome), a potential emergency needing immediate intervention, whereas less than 10% of them appear with hypovolemic shock.
Renal AML is usually benign and a true malignant AML is rare.
Risk factors for malignant AML include size >7 cm, tumor necrosis, and epithelioid carcinoma-like pattern.
Is this image adequate?
No.
CT with contrast to confirm the benign tumor
MRI to detect fat content and also evaluate the other kidney.
What are the other diseases associated with angiomyolipoma?
It may be association with the tuberous sclerosis complex (TSC) in about 20%–30%
can be also associated lymphangiolieymatosis.
References
Anton DG, Kovvuru K, Kanduri SR, Aeddula NR, Bathini T, Thongprayoon C, Kaewput W, Wijarnpreecha K, Watthanasuntorn K, Salim SA, Matemavi P, Vaitla P, Rivera FC, Cheungpasitporn W. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urol Ann. 2021 Jan-Mar;13(1):67-72.
1- yes
2- It is a benign (non cancer) tumor of fat and muscle tissue that is usually foind in the kidney
3- It may enlarge and cause pain and bleeding
It may cause renal failure and UTI and anemia
4- yes, diagnosis can be made using CT scan, ultrasound or MRI
Yes, because the size of this Angiomyolipomas (AML) is limitrofe to small-asymptomatic and we can still evaluate if we use mTOR which will help to control its development.
Angiomyolipomas (AML) of the kidney are non-encapsulated benign neoplasms composed of thickened vessels (that lack normal elastic tissue), smooth muscle and adipose tissue that arise from the mesenchymal elements of the kidney
The greatest risks are related to bleeding, mainly retroperitoneal
Yes, it is better to use a more accurate image exam to evaluate the size and borders (CT or NMR) and to follow up with USG.
The others more commom are:
– Tuberous sclerosis complex (TSC),
– Pulmonary lymphangioleiomyomatosis(LAM),
– Epithelioid variant of AML
REFERENCE:
– Gopalakrishnan N, Dhanapriya J, Sakthirajan R, Dineshkumar T, Balasubramaniyan T, Haris M. Angiomyolipoma of donor kidney: Successful transplantation and 5-year follow-up. Indian J Nephrol. 2016 Mar-Apr;26(2):131-3. doi: 10.4103/0971-4065.159552. PMID: 27051138; PMCID: PMC4795429.
– Anton DG, Kovvuru K, Kanduri SR, Aeddula NR, Bathini T, Thongprayoon C, Kaewput W, Wijarnpreecha K, Watthanasuntorn K, Salim SA, Matemavi P, Vaitla P, Rivera FC, Cheungpasitporn W. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urol Ann. 2021 Jan-Mar;13(1):67-72. doi: 10.4103/UA.UA_14_20. Epub 2021 Jan 19. PMID: 33897168; PMCID: PMC8052899.
Q1: yes, if this is a unilateral AML in this half-haplotype donor with DSA.
The affected kidney is considered for donation after ex-vivo excision of the AML.
But bilateral large AMLs are not suitable for donation.
Q2-: AML is a benign tumor with adipose tissue in addition to smooth muscle and blood vessels.
It is important to differentiate benign AML from epithelioid AML which may become malignant.
Q3: The most important risk is hemorrhage that even may result in shock.
Another risk which should considered is risk of malignancy transmission
Q4: No, bilateral kidney imaging is needed to exclude bilateral involvement that is not considered for donation.
Q5:
· Tuberous sclerosis disease
· Neurofibromatosis type 1
·
lymphangioleiomyomatosis
References:
1. Andrews, P. A., & Burnapp, L. (2018). British Transplantation Society / Renal Association UK guidelines for living donor kidney transplantation 2018: Summary of updated guidance. In Transplantation (Vol. 102, Issue 7). https://doi.org/10.1097/TP.0000000000002253
Will you accept this donor?
Yes, I will accept this donor as long as the mass is unilateral and resect-able.
What is angiomyolipoma?
AML is a benign tumor that arise from mesenchymal tissue of the kidney and formed of thick-walled blood vessels, smooth muscle cells and mature adipose tissue
What are the risks involved?
Risks include: pain, rupture, bleeding, hypovolemic shock, malignant transformation especially in TS patients and pregnant women.
Is this image adequate?
The image isn’t adequate because it shows only one kidney, proper scan is needed to check if there is bilateral kidney involvement.
What are the other diseases associated with angiomyolipoma?
AML can be encountered in patients with TS, NF-1, Von-Hipple Lindau disease and Lymphangio-leio-myo-matosis.
References:
British Transplantation Society. Renal Association Guidelines for Living Donor Kidney Transplantation, 4th ed.; British Transplantation Society: Macclesfield, UK, 2018; Available online: https//bts.org.uk/wp-content/uploads/2018/07/FINAL_LDKT-guidelines_June-2018.
Usually AML is benign lesion found accidentally
we should do MRI and DTPA before proceed
I will accept this donor and took the kidney with this lesion after the excision of AML during the transplant operation
Will you accept this donor?
Yes I will accept this donor provided the Angiomyolipoma is unilateral. Depending on the size of the lesion (>1 cm) would consult with transplant surgeon for removal during surgery.
What is angiomyolipoma?
As the name implies it is a tumor having blood vessels, smooth muscle and adipose tissue. It is usually benign in nature.
What are the risks involved?
· Bleeding is the most common complication. Severe events leading to shock and renal function impairment can occur
· Association with Tuberous sclerosis complex.
· Malignant transformation is rare but can occur in epitheloid variant of AML
Is this image adequate?
This image is inadequate. Ideal imaging technique for Angiomyolipoma is contrast MRI, however Contrast enhanced CT can suffice for diagnostic purposes. CT should be 3-5 mm slices and include both kidneys to rule out tuberous sclerosis and other differentials.
What are the other diseases associated with angiomyolipoma?
· Tuberous sclerosis complex
· Lymphangioleiomyomatosis
REFERENCES:
· Anton DG, Kovvuru K, Kanduri SR, et al. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urol Ann. 2021;13(1):67-72. doi:10.4103/UA.UA_14_20
· BT/RA Living Donor Kidney Transplantation Guidelines 2018
MRI is needed for confirmation ,or even CT guided biopsy.
The kidney with AML can be donated after removal of AML as immunosuppression will increase possibility of malignancy transformation; leaving the normal kidney for the donor.
Rofaiel, George et al. “Successful Utilization of a Live Donor Kidney with Angiomyolipoma.” Cureus vol. 12,2 e6937. 10 Feb. 2020,
Will you accept this donor:
As AML >4 cm generally precludes living kidney donation, but unilateral large (>4 cm) AML can be used if ex vivo excision of the AML appears to be straightforward.
If there is no contraindication i will accept this donor
What is angiomyolipoma:
Angiomyolipoma is a benign tumour ,leading to excessive growth of hamartoma lesion, either sporadic or associated with TSC.
AMLs have been reported in 0.13 to 2.2 percent of asymptomatic adults who underwent imaging
What are the risks involved:
Hemorrhage often results in significant pain, anemia, and sometimes, hemorrhagic shock.
AMLs that are larger than 6 cm have a higher likelihood of hemorrhage.
Wunderlich syndrome,
Renal AMLs are the most common cause of Wunderlich syndrome, a life-threatening, nontraumatic kidney haemorrhage into the subcapsular and perirenal spaces.
Is this image adequate:
MRI could be more sensitive than CT in fat-poor AMLs.
If the diagnosis is still uncertain by MRI or CT, an image-guided percutaneous core needle biopsy can be done.
What are the other diseases associated with angiomyolipoma:
AML is benign tumor of the kidney composed mainly of lipid. unless getting bigger quickly usually just follwed up conservatively.
It is the second reason for bleeding after RCC. There are scarece case reports for tranplanting kidney with. AML . In a case report (1) an ex-vivo resection was successefly removed before transplantaton.
Based on that and on importance of utilization of available donated kidney , it is wise to accept this donor.
The image is adequate. CT or MRI with contrast differentiate AML from RCC.
Can occur sporadically (80%) or can be associated with certain hereditary conditions (20%) like tuberous sclerosis and pulmonary lymphangioleiomyomatosis (2)
—————
1- https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7067347/
2-https://www.ncbi.nlm.nih.gov/books/NBK585104/
111 HLA mismatch & no DSA. CT scan showed a large (4.6cm) angiomyolipoma. Before accept this donor need evaluation of angiomyolipoma. Is this tumour is sporadic or associated with other disease. In case of sporadic angiomyolipoma i will accept this donor for renal transplantation
This is a benign tumour consists of smooth muscle cell, fat & thick vessel.
a) High risk of retroperitoneal haemorrhage if tumour is associated with
– Large size (>6cm)
– Large intratumour aneurysm (>5cm)
– Pregnancy / Hormone therapy
– Uncontrolled HTN
– Anticoagulant drug
b) Malignant transformation (i.e sarcoma) is associated with tuberous sclerosis.
No, this image is not adequate, need image of the contralateral kidney. Also require CT/ MRI of brain.
– Tuberous sclerosis
– Lymphangioleiomyomatosis
From immunological wise I will accept the donor as the MM is 1 – 1 – 1 , about the donor AML its unilateral , incidentally finding , a symptomatic , not associted with TSC, pulmonary LAM, or epithelioid variant of AML , but because the size of lesion is more than 4 cm should do ex vivo excision .
Angiomyolipomas (AMLs) are benign neoplasms occurring in the kidney and are composed of varying amounts of tissue resembling blood vessels, smooth muscles, and adipose. The vast majority of AMLs are benign but malignant transformation has been reported .
Renal AMLs may occur sporadically or in association with either tuberous sclerosis complex (TSC), sporadic lymphangioleiomyomatosis (LAM), or a combination (TSC-LAM).
1) vascular complications and retroperitoneal hemorrhage with tumors >4 cm
2) malignant transformation : epithelioid variants, which sometimes undergo malignant transformation, are “fat-poor” or “fat-invisible” by imaging.
3) risk of bleeding : bleeding from renal AMLs can be mild or catastrophic resulting in hemorrhagic shock, loss of function of the affected kidney, and death [6,7]. The risk of bleeding increases with the size of the AML and the growth of the vasculature within it. We evaluate bleeding risk with imaging
Yes , CT is the modality of choice to diagnose AML
TSC , LAM
Reference :
1)https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8052899/#:~:text=Renal%20angiomyolipoma%20(AML)%20is%20the,has%20not%20been%20well%20established.
2) https://pubmed.ncbi.nlm.nih.gov/33897168/
3) Up to date
46 yr male renal donor for his brother; haplo-match
4.6cm heterogenous non-enhencing exophytic mass (AML) in left kidney
Yes – the donor being well matched to recipient is acceptable. Risk of malignancy with small AML <5cm is negligible – but the lesion shall be enucleated on bench with reconstruction (Renorhaphy).
· What is angiomyolipoma?
AML – as per the name on histologic features, is a benign slow growing hamartoma lesion composed of abnormal, thick-walled vessels, with varying amounts of smooth muscle like cells and adipose tissue.
Sporadic cases are rare (0.13 -1% of general population) and asymptomatic. These are the most common renal lesions in patients with TSC, evident in approximately 80% of TSC patients by age 10 years.
asymptomatic for many years, slow growing (2-5mm/year)
Large mass – may present with flank pain, bleeding.
Rare cases of malignant transformation in AML and coexistence of Adeno Ca been reported.
Sporadic Primary AML – mostly asymptomatic for many years –
Flank pain (large size >6cm), bleed (spontaneous renal haemorrhage, perinephric hematoma.
may have haematuria, hypertension, anaemia, and kidney function impairment if very large, mass effect. Usually does not recur after excision.
TSC & VHL associated – multiple, bilateral and recurrent
Malignant transformation – very rarely (angiosarcoma, Epithelioid Malignant AML; Adeno carcinoma coexistent).
The literature review of malignant EAML led to the identification of 8 malignant features: size ≥5 cm; metastasis; infiltration; necrosis; ≥50% atypical epithelioid cells; cytologic atypia; atypical mitosis; and vessel invasion. The co-existence of at least 5 of these is proposed to indicate malignant EAML.
Risk in the donor after donation of left kidney bearing AML – No risk, as the right kidney is normal.
Risk in Recipient: after ex-vivo complete excision of lesion on bench + reconstruction – minimal risks of bleeding / subcapsular hematoma, some areas of necrosis, urine leakage and urinoma, infection.
Risk of recurrence (if incomplete excision / residual tissue / angioinvasion) and rarely malignant transformation.
· Is this image adequate?
No
CE MRI would delineate a clear outline and necrosis (if any)
CT Renal Angio + delayed image with IVU with 3D reconstruction – will give a complete picture of feeding vessels, other vascular anatomy and relation to Pelvi-caliceal anatomy.
· What are the other diseases associated with angiomyolipoma?
TSC –
VHL –
1. References:
Siroky BJ, Czyzyk-Krzeska MF, Bissler JJ. Renal involvement in tuberous sclerosis complex and von Hippel-Lindau disease: shared disease mechanisms? Nat Clin Pract Nephrol. 2009;5(3):143–156
2. Kawaguchi, Ken-ichi; Oda, Yoshinao; Nakanishi, Kazuo, et al. Malignant Transformation of Renal Angiomyolipoma – A Case Report. The American Journal of Surgical Pathology 2002 April; 26: 523-529.
3. Brimo F, Robinson B, Epstein J, et al. Renal Epithelioid Angiomyolipoma with Atypia: a Series of 40 Cases With Emphasis on Clinicopathologic Prognostic Indicators of Malignancy. The American Journal of Surgical Pathology 2010 May.
4. Ons Boudaouara , Rim Kallel, Dhouha Dhieb , et al. Renal angiomyolipoma: Clinico-pathologic study of 17 cases with emphasis on the epithelioid histology and p53 gene abnormalities. Ann Diagnostic Pathology 2020 Aug; 47:151538
Ragheed Saoud , Tanya W Kristof , Clark Judge , et al. Clinical and pathological features of renal epithelioid angiomyolipoma (PEComa): A single institution series. Urol Oncol 2022 Feb;40(2):18-24.
46 yr male renal donor for his brother; haplo-match
4.6cm heterogenous non-enhencing exophytic mass (AML) in left kidney
Yes – the donor being well matched to recipient is acceptable. Risk of malignancy with small AML <5cm is negligible.
· What is angiomyolipoma?
AML – as per the name on histologic features, is a benign slow growing hamartoma lesion composed of abnormal, thick-walled vessels, with varying amounts of smooth muscle like cells and adipose tissue.
Sporadic cases are rare (0.13 -1% of general population) and asymptomatic. These are the most common renal lesions in patients with TSC, evident in approximately 80% of TSC patients by age 10 years.
asymptomatic for many years, slow growing (2-5mm/year)
Large mass – may present with flank pain, bleeding.
Rare cases of malignant transformation in AML and coexistence of Adeno Ca been reported.
Sporadic Primary AML – mostly asymptomatic for many years –
Flank pain (large size >6cm), bleed (spontaneous renal haemorrhage, perinephric hematoma.
may have haematuria, hypertension, anaemia, and kidney function impairment if very large, mass effect. Usually does not recur after excision.
TSC & VHL associated – multiple, bilateral and recurrent
Malignant transformation – very rarely (angiosarcoma, Epithelioid Malignant AML; Adeno carcinoma coexistent).
The literature review of malignant EAML led to the identification of 8 malignant features: size ≥5 cm; metastasis; infiltration; necrosis; ≥50% atypical epithelioid cells; cytologic atypia; atypical mitosis; and vessel invasion. The co-existence of at least 5 of these is proposed to indicate malignant EAML.
Risk in the donor after donation of left kidney bearing AML – No risk, as the right kidney is normal.
Risk in Recipient: after ex-vivo complete excision of lesion on bench + reconstruction – minimal risks of bleeding / subcapsular hematoma, some areas of necrosis, urine leakage and urinoma, infection.
Risk of recurrence (if incomplete excision / residual tissue / angioinvasion) and rarely malignant transformation.
· Is this image adequate?
No
CE MRI would delineate a clear outline and necrosis (if any)
CT Renal Angio + delayed image with IVU with 3D reconstruction – will give a complete picture of feeding vessels, other vascular anatomy and relation to Pelvi-caliceal anatomy.
· What are the other diseases associated with angiomyolipoma?
TSC –
VHL –
1. References:
Siroky BJ, Czyzyk-Krzeska MF, Bissler JJ. Renal involvement in tuberous sclerosis complex and von Hippel-Lindau disease: shared disease mechanisms? Nat Clin Pract Nephrol. 2009;5(3):143–156
2. Kawaguchi, Ken-ichi; Oda, Yoshinao; Nakanishi, Kazuo, et al. Malignant Transformation of Renal Angiomyolipoma – A Case Report. The American Journal of Surgical Pathology 2002 April; 26: 523-529.
3. Brimo F, Robinson B, Epstein J, et al. Renal Epithelioid Angiomyolipoma with Atypia: a Series of 40 Cases With Emphasis on Clinicopathologic Prognostic Indicators of Malignancy. The American Journal of Surgical Pathology 2010 May.
4. Ons Boudaouara , Rim Kallel, Dhouha Dhieb , et al. Renal angiomyolipoma: Clinico-pathologic study of 17 cases with emphasis on the epithelioid histology and p53 gene abnormalities. Ann Diagnostic Pathology 2020 Aug; 47:151538
5. Ragheed Saoud , Tanya W Kristof , Clark Judge , et al. Clinical and pathological features of renal epithelioid angiomyolipoma (PEComa): A single institution series. Urol Oncol 2022 Feb;40(2):18-24.
British Transplantation Society. Renal Association Guidelines for Living Donor Kidney Transplantation, 4th ed.; British Transplantation Society: Macclesfield, UK, 2018; Available online: https//bts.org.uk/wp-content/uploads/2018/07/FINAL_LDKT-guidelines_June-2018.pdf
DONOR WITH AML IS A MARGINAL DONOR AND CAN BE ACCEPTED IF ANY OTHER DONOR IS NOT AVAIALBE AND FAMILY ACCEPT THE RISK
AML IS BENIGN LESION OF HIGH VASCULARITY AND RISK OF BLEEDING IS HIGH IF SIZE IS MORE THAN 4 CM
NEED TO RULE OR TUBEROUS SCLEROSIS IN DONOR WITH AML BY HISTORY OF SEIZURES , SKIN LESION AND FAMILY HISTORY , MRI BRAIN
RISK OF SPONTANEPOUS BLEEDING IS HIGH AS THE SIZE OF AML INCREASES AFTER 4 CM
NO RISK OF METATSASIS AS IT IS BENIGN LESION
MORE IMAGING IN THE FORM OF RENAL ANGIOGRAPHY IS REQUIRED TO SEE IF ANY RENAL ANEURYSM IS SEEN
SPIT RENAL FUNCTIONS LIKE DTPA RENOGRAM
ANGIOGRAPHY IS ALSO HELPFULL IN PLANNING ANGIOEMBOLISATION
IF AT ALL I AM LEFT WITH NO CHOICE BIT TO ACCEPT THIS KIDNEY , I WILL DO SUPERSELECTIVE ANGIOEMBOLISATION BEFORE THE KIDNY DONATION AND THEN WILL DO THE TRANSPLANT
OTEHR OPTION IS TO DO EN BLOC EXCISION ON BENCH AND DO TRANSPLANT
AML IS ASSOCIATED WITH RENAL ARERY ANEURYSM AND TUBEROUS SCLEROSIS
Yes, i accept because 4,6cm angiomyolipoma is not a contra indication.
AMLs are benign renal hamartomas composed of abnormal, thick-walled vessels and varying amounts of smooth muscle–like cells and adipose tissue.
These are the most common renal lesions in patients with TSC, evident in approximately 80% of TSC patients by age 10 years.
Whereas solitary AMLs are found in the general population, particularly among older women.
Hemorrhage especially if more than 6cm, pain, anemia, and sometimes, hemorrhagic shock, in cases of TSC involving other organs.
Yes
VHL
TSC
Reference:
Siroky BJ, Czyzyk-Krzeska MF, Bissler JJ. Renal involvement in tuberous sclerosis complex and von Hippel-Lindau disease: shared disease mechanisms? Nat Clin Pract Nephrol. 2009;5(3):143–156
UpToDate.
AML >4 cm generally precludes living kidney donation, but unilateral large (>4 cm) AML can be used if ex vivo excision of the AML appears to be straightforward
It is a neoplasm occurring in the kidney that is composed of varying amounts of tissues resembling blood vessels, smooth muscle, and adipose tissue.
AMLs have been reported in 0.13 to 2.2 percent of asymptomatic adults who underwent imaging
The vast majority of renal AMLs cause no symptoms.
Among symptomatic patients, features related to hemorrhage appear to be the most common. These include abdominal or flank pain (associated with the mass effect of the hemorrhage) and tenderness, hematuria, hypertension, anemia, and kidney function impairment
Hemorrhage often results in significant pain, anemia, and sometimes, hemorrhagic shock.
AMLs that are larger than 6 cm have a higher likelihood of hemorrhage.
Wunderlich syndrome
Renal AMLs are the most common cause of Wunderlich syndrome, a life-threatening, nontraumatic kidney hemorrhage into the subcapsular and perirenal spaces
MRI could be more sensitive than CT in fat-poor AMLs.
Renal AMLs may either occur sporadically or in association with either tuberous sclerosis complex (TSC), sporadic lymphangioleiomyomatosis (LAM), or a combination (TSC-LAM).
Will you accept this donor?
if no other contra-indications ….yes I accept
What is angiomyolipoma?
bengin tumor ,leading to excissive growth of hamartoma lesion, either sporadic or associated with TSC
What are the risks involved?
in cases of TSC , associated with multifocal, bilateral and involving other organs .
if large may associated with spontaneous rupture (wunderlich syndrome)
Is this image adequate?
yes but better discussed in radiology revision meeting . if any doubt ….MRI
What are the other diseases associated with angiomyolipoma?
4,6cm angiomyolipoma is not a contra indication. As per British Transplantation Society advised:
Bilateral AML and AML >4 cm generally preclude living kidney donation
although occasionally unilateral large (>4 cm) AML can be used if ex
vivo excision of the AML appears to be straightforward.
An incidental, unilateral solitary AML <4 cm with typical characteristic
CT criteria does not usually preclude donation.
A kidney with an AML <1 cm may be considered for a donation or left in
situ in the donor’s remaining kidney.
Kidneys containing a single AML between 1 and 4 cm can be
considered for donation depending on its position, consideration of
whether ex vivo excision of the AML is straightforward, or whether it can be left in situ in the recipient and followed with serial ultrasound imaging.
Renal AML is a heterogeneous, triphasic tumor with varying elements of smooth muscle, adipose tissue, and vascular elements. Renal AML can occur sporadically with an incidence ranging from 55% to 80%[ or in association with the tuberous sclerosis complex (TSC) in about 20%–30% and very rarely as sporadic lymphangioleiomyomatosis (LAM).
Risk factors for malignant AML include:
1-size>7 cm.
2-Tumor necrosis.
3-Epithelioid carcinoma‑like pattern. The historical criteria for active intervention of
renal AML are symptomatic lesions >4 cm with risk of rupture, intolerable pain, hemorrhagic hypovolemic shock, suspected malignancy, especially in renal AML
Imaging of the other kidney is needed as it will affect the decision of donation.
a-b Renal AML can occur sporadically with an incidence ranging from 55% to 80%[10] or
b-In association with the tuberous sclerosis complex (TSC) in about 20%–30% and very
c-Rarely as sporadic lymphangioleiomyomatosis (LAM).
References:
1-Dr. Wisit Cheungp, Published: 19.01.2021. 2021 Urology Annals
Will you accept this donor?
Yes, donation should only be contemplated if excision of the AML is possible ex-vivo.
What is Angiomyolipoma?
Renal angiomyolipoma (AML) is the most frequent mesenchymal tumor of the kidney.
Its histopathology was originally described by Fischer in 1911.
Renal AML is a heterogeneous, triphasic tumor with varying elements of smooth muscle, adipose tissue, and vascular elements.
Renal AML can occur sporadically with an incidence ranging from 55% to 80% or in association with the tuberous sclerosis complex (TSC) in about 20%–30% and very rarely as sporadic lymphangioleiomyomatosis (LAM).
What are the risks involved?
a) Hemorrhage
b) Malignancy changes
c) In female there is risk of rupture during pregnancy
Is this image adequate?
No, we need to see both the kidneys. MRI abdomen is very sensitive in diagnosing and distinguishing fat-poor AML lesions from renal malignancies. We also need to take suggestion of a specialist uroradiologist.
What are the other diseases associated with angiomyolipoma?
1) Tuberous sclerosis
2) Lymphangieoleiomyomatosis
3) Von hippel landau disease
4) Neurofibromatosis type 1
References :
1-Anton, Desiree Garcia; Kovvuru, Karthik; Kanduri, Swetha R; Aeddula, Narothama Reddy1; Bathini, Tarun2; Thongprayoon, Charat3; Kaewput, Wisit4; Wijarnpreecha, Karn5; Watthanasuntorn, Kanramon6; Salim, Sohail Abdul; Matemavi, Praise7; Vaitla, Pradeep; Rivera, Franco Cabeza; Cheungpasitporn, Wisit. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urology Annals 13(1):p 67-72, Jan–Mar 2021. | DOI: 10.4103/UA.UA_14_20
2-Nalesnik MA, Woodle ES, Dimaio JM, et al. Donor-transmitted malignancies in
organ transplantation: assessment of clinical risk. Am J Transplant 2011; 11:
1140-7.
I will accept the donor. 2018 systematic fourteen studies with a total of 16 donors with AML (without an associated diagnosis of tuberous sclerosis, lymphangiomyomatosis or epitheloid variant) did not reveal an increased risk for both the donor and recipient. None of the donors with AML progressed to end-stage kidney disease or died at during and none of the recipients had malignant transformation of AML during the follow up period.
Angiomyolipoma is a kidney tumour characterized by an outgrowth comprising of smooth muscles, blood vessels and adipose. It is mostly benign but can undergo malignant transformation especially the epitheloid variant. It can be sporadic or associated with tuberous sclerosis or lymphangioleiomyomatosis. Its predominantly affects females.
It is benign but can transform to cancer. It can bleed resulting in hematuria, acute kidney injury. The tumor infiltration can lead to renal impairment and hypertension.
MRI with gadolinium is better to determine vascularity and the amount of fat present which will determine approach to treatment.
Tuberous sclerosis and lymphangioleiomyomatosis.
Reference
Anton DG et al. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urol Ann. 2021
Up to date
Will you accept this donor?
Yes, unilateral AML is possible to donate. I will maintain the normal kidney to the donor and donate the one with AML
What is angiomyolipoma?
It is a benign vascular neoplasm, predominantly composed of fat
What are the risks involved?
Malignancy
Bleeding
Is this image adequate?
We must review the other kidney. MRI should be done
What are the other diseases associated with angiomyolipoma?
Neurofibromatosis type 1
Lymphangioleiomyomatosis
Tuberous Sclerosis
Von Hippel Lindau
Will you accept this donor?
So, I would accept this patient if it is benign, fat rich AML and possible for surgical excision and I would like to transplant AML kidney for the recipient.
What is angiomyolipoma?
Renal AML is a heterogeneous, triphasic tumor with varying elements of smooth muscle, adipose tissue, and vascular elements
What are the risks involved?
Risk factors for malignant AML
Risks involved
Is this image adequate?
I would like to proceed with MRI to get more information if its fat poor AML.
What are the other diseases associated with angiomyolipoma?
References:
– Will you accept this donor?
– I would accept this donor if unilateral, isolated & resectable AML
– The affected kidney will be taken
– What is angiomyolipoma?
– AML is the most common mesenchymal tumor of the kidney. composed of fat, vascular & smooth muscle cells.
– What are the risks involved?
– Risk of Hemorrhage, malignancy transformation to sarcoma.
– Is this image adequate?
– Need CT imaging of the other kidney.
– What are the other diseases associated with angiomyolipoma?
– Tuberous Sclerosis
– Von Hippel Lindau
– Neurofibromatosis type 1
– Lymphangioleiomyomatosis
Will you accept this donor?
-I will accept if confirmed unilateral AML , especially if it is the only available donor with pre-transplant excision of the tumor (in-vivo or ex-vivo), and the lesion (4.6cm) can be resected before implantation
-If Confirmed Unilateral AML after Repeated MRI or CT w contrast Abdomen for both kidneys
-If bilateral AML, not respectable and the lesion is with rapid rate of growth on monitoring => i will decline the donor
What is angiomyolipoma?
Angiomyolipoma (AML) is a benign neoplasm non-encapsulated consist of mature adipose tissue, smooth muscle and thick-walled blood vessels, arise from mesenchymal tissue of the kidney and can diagnosed as an incidental finding in donor work-up
•What are the risks involved?
Hge, from ruptured blood vessels or aneurysm(most common)
Pain, (abdominal or flank pain )
Hypovolemic /Hgic. Shock,
Malignancy
•Is this image adequate?
-No, there is a need to see both kidneys for proper evaluation of the patient.
-CT multidetector ,MRI with contrast of the other side and Hx and general physical examination of patient for features of TSC.
What are the other diseases associated with angiomyolipoma?
-Tuberous Sclerosis Complex
– VHL (Von Hippel-Lindau disease)
– Neurofibromatosis type 1
– Sporadic Lyphangioleiomyomatosis
Reference:
British Transplantation Society. Renal Association Guidelines for Living Donor
Kidney Transplantation, 4th ed.; British Transplantation Society: Macclesfield, UK,
2018; Available online: https//bts.org.uk/wp content/uploads/2018/07/FINAL_LDKT-guidelines_June-2018.pdf (accessed on 30 January 2023).
Vicente E Torres, and York Pei. Renal angiomyolipomas (AMLs): Management. © 2023
UpToDate (accessed on 3 February 2023).
Anton DG, Kovvuru K, Kanduri SR, et al. Use and outcomes of kidneys from donors with
renal angiomyolipoma: A systematic review. Urol Ann. 2021 Jan-Mar;13(1):67-72
BT/RA Living Donor Kidney Transplantation Guidelines 2018
UpToDate
That is a good summary and an interesting analysis. Please type headings and sub-headings in bold or in underline.
· Will you accept this donor?
. If I say about my own center we do not proceed, otherwise, literature say when the AML >4 cm or bilateral needs surgical removal. If less than 2 cm or unilateral wait and watch with good follow-up every three month
. There reported cases with liver transplantation and five year survival free without tumor.
. There are reported cases reassuring for safety of donor with AML as well as recipient without any progression of disease, conversion into malignancy and tuberous sclerosis.
. So if small lesion can proceed for donation safely after excision.
· What is angiomyolipoma?
· The Angiomyolipoma are composed of mature fat appearing, thick wall, and poorly organized neoplasm of varying blood vessels, adipose tissue, and smooth muscle cells. The tumor of kidney and majority are benign, very rarely they become malignant.
· They can occur as only with in the kidney and sometime associated with tuberous sclerosis complex. There are other histological variations, 1. Classic, 2. Epitheliod, 3. Very rare cystic variant.
· This is a very rare disease reported <2%.
· More common in females, may female sex hormones promote the growth of renal AMLs.
· This is non-encapsulated benign neoplasm with incidence 45-80% association with tuberous sclerosis.
·
· What are the risks involved?
. Size of tumor at diagnosis,
. Female gender,
. Hemorrhage,
. Malignant transformation.
· Is this image adequate?
. Further second kidney imaging needed,
. CT with contrast chest, abdomen and pelvis for metastasis and lymphadenopathy,
. Tumor markers. .
· What are the other diseases associated with angiomyolipoma?
. AML variants,
. Sporadic AML,
. Tuberous sclerosis,
. Tuberous sclerosis complex,
. RCC.
. VHL.
. Refrences:
1.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6007575/
2. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4795429/
3. https://www.infona.pl/resource/bwmeta1.element.elsevier-7297f87d-e288-33fd-8387-60c6b36f1d5b/tab/citations.
4. https://journals.lww.com/urol/Fulltext/2021/13010/Use_and_outcomes_of_kidneys_from_donors_with_renal.13.aspx.
That is a good summary and an interesting analysis.
I will accept such donor if the other kidney free and anatomically normal
According to BTS/RA Living Donor Kidney Transplantation Guidelines 2018 recommendations
>> Bilateral AML and AML >4 cm generally preclude living kidney donation although occasionally unilateral large (>4 cm) AML can be used if ex vivo excision of the AML appears to be straightforward.
>> An incidental, unilateral solitary AML <4 cm with typical characteristic CT criteria does not usually preclude donation.
>> A kidney with an AML <1 cm may be considered for donation or left in situ in the donor’s remaining kidney.
>> Kidneys containing a single AML between 1 and 4 cm can be considered for donation depending on its position, consideration of whether ex vivo excision of the AML is straightforward, or whether it can be left in situ in the recipient and followed with serial ultrasound imaging.
Angiomyolipomas (AMLs) are neoplasms occurring in the kidney that are composed of varying amounts of tissues resembling blood vessels, smooth muscle, and adipose tissue. The vast majority of AMLs are benign but malignant AMLs have been reported.
AMLs have been reported in 0.13 to 2.2 percent of asymptomatic adults who underwent imaging.
Renal AMLs appear to be more common among females.
hemorrhage appear to be most common risk . The risk of significant hemorrhage is related to the size of the AMLs, degree of vascularity, the size of aneurysms within the AML, and the growth pattern (exophytic growth is associated with a higher risk than endophytic growth) .
AMLs that are larger than 6 cm have a higher likelihood of hemorrhage Increased vascularity and/or an intralesional aneurysm that is 5 mm or larger are also associated with an increased risk of hemorrhage .
Pregnancy increased the risk of hemorrhage.
Hemorrhage is the most feared clinical complication of AMLs due to its severe nature, often resulting in significant pain, anemia, and sometimes, hemorrhagic shock
Renal AMLs are the most common cause of Wunderlich syndrome, a life-threatening, nontraumatic kidney hemorrhage into the subcapsular and perirenal spaces .
abdominal or flank pain (associated with mass effect of the hemorrhage) and tenderness, hematuria, hypertension, anemia, and kidney function impairment .
No , we should review the other kidney
Contrast enhanced renal CT scan, ultrasound and / or MRI can usually distinguish between benign lesions such as angiomyolipoma (AML) or malignancy such as renal cell carcinoma (RCC). Review by a specialist uroradiologist is recommended.
>> tuberous sclerosis complex (TSC)
>> sporadic lymphangioleiomyomatosis (LAM)
>> combination (TSC-LAM)
Reference
BTS/RA Living Donor Kidney Transplantation Guidelines 2018
Uptodate Renal angiomyolipomas (AMLs): Epidemiology, pathogenesis, clinical manifestations, and diagnosis
That is a good summary and an interesting analysis. Please type headings and sub-headings in bold or in underline.
Will you accept this donor?
Yes, can proceed with transplantation with AML kidney and ex-vivo lesion removal of this single lesion, sparing lesion free kidney for donor.
What is angiomyolipoma?
Lesions in the kidney comprised of smooth-muscle-like cells, adipocyte-like cells, and epithelioid cells. The three cell types appear to be derived from pericytes. It could be sporadic or associated with Tuberus sclerosis complex. AML could be of classic variant, Epithelioid variant or AML with epithelial cysts. In one study it was found in 2.2 percent of healthy donors.
What are the risks involved?
· Malignant transformation
· Hemorrhage
Is this image adequate?
CT with contrast of the other side and Hx and general physical examination of patient for features of TSC.
What are the other diseases associated with angiomyolipoma
· Tuberous Sclerosis Complex
· VHL
· Neurofibromatosis
· Sporadic Lyphangioleiomyomatosis
References; Uptodate Topic 131412 Version 5.0
That is a good summary and an interesting analysis.
Will you accept this donor?
—————————————————–
Before accepting this donor,need detailed CT scan Abdomen to rule out bilateral disease.If unilateral ,then can be accepted for donation after excision.However,if bilateral with the mentioned size then can not be considered for donation.(BTS guidelines)
What is angiomyolipoma?
Angiomyolipomas (AMLs) can be benign or malignant and composed of smooth muscles,blood vessels and adipose tissue.It has female predominance,and can be unilateral or bilateral with rich in fat or poor in fat or fat invisible .
What are the risks involved?.
Risk of rupture is high if aneurysms >5 mm or the size of the tumor is larger than 6 cm or if the female is pregnant or taking hormonal therapy.Other risks which are associated with this include malignant transformation, infection and hypertension.
Is this image adequate?
No, imaging not adequate as bilateral images needed as few diseases have bilateral involvement and also MRI with gadolinium is more sensitive in cases of fat poor lesions. Other scans also needed like CT scan Chest to rule out LAM
What are the other diseases associated with angiomyolipoma?
Tuberous sclerosis complex (TSC).
Sporadic lymphangioleiomyomatosis
Neurofibromatosis type 1
Von Hippel-Lindau disease
REFERENCES:
1-Anton DG, Kovvuru K, Kanduri SR, et al. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urol Ann. 2021 Jan-Mar;13(1):67-72
2-BT/RA Living Donor Kidney Transplantation Guidelines 2018
That is a good summary and an interesting analysis.
Yes, I will accept this donor
It’s benign condition as collection of fat and muscle tissue found accidentally by imaging investigations
Risk of enlargement lead to pain and may grow to renal failure and also risk of bleeding
MRI is confirmatory
Genetic disease like tuberous sclerosis
That is a good summary and an interesting analysis. Can you upload some evidence please
Will you accept this donor?
Not easy to answer, it needs full assessment and MDT discussion and exploring other straight forward options like other donors or DCD options.
What is angiomyolipoma?
Angiomyolipomas (AMLs) are benign neoplasms occurring in the kidney and are composed of varying amounts of tissue resembling blood vessels, smooth muscles, and adipose.
What are the risks involved?
Malignancy, bleeding
Is this image adequate?
MRI is the modality of choice
What are the other diseases associated with angiomyolipoma?
Renal AMLs may occur sporadically or in association with either tuberous sclerosis complex (TSC), sporadic lymphangioleiomyomatosis (LAM), or a combination (TSC-LAM).
Resources:
That is a good summary and an interesting analysis
Will you accept this donor?
-Yes,I will accept him .In unilateral disease, generally only the affected kidney should be considered for donation and if excision of the AML is possible, because of the risk of subsequent symptoms.
What is angiomyolipoma?
-Classic angiomyolipoma (AML) is a triphasic, benign neoplasm composed of mature adipose tissue, smooth muscle and thick walled blood vessels and can occur as an incidental finding in donor work-up.
What are the risks involved?
AML more than 4 cm in diameter at presentation were more likely to
have significant growth on long term follow up. it can rupture and bleed.
Is this image adequate?
Diagnosis of an AML can usually be made by imaging without recourse to biopsy; however, it is important to discriminate classic AML from the uncommon subtype of epitheloid AML, which may have a malignant phenotype. A specialist uroradiologist should review all cases.
What are the other diseases associated with angiomyolipoma?
-Tuberous sclerosis
-They are also commonly found in women with the rare lung disease lymphangioleiomyomatosis.
Reference:
-Living Donor Kidney Transplantation Guidelines 2018.Fourth Edition. British Transplantation Society http://www.bts.org.uk .March 2018
That is an interesting analysis. I like your decision-making process in accepting this kidney.
Will you accept this donor?
Yes, i can accept, as AML is a benign mass on the kidney, especially in female, and its rarely reported as cases of transformation.
What is AML:
AML is a benign kidney mass mostly formed from fatty tissue, smooth muscles and blood vessels.
Accounted about 1% of all resected kidney tumors.
They are usually single and unilateral, but multiple and bilateral although less common can occur.
Risks:
Is the image adequate:
Although not strongly recommended, (if feasible) needle biopsy can be done if there is doubt about diagnosis.
Diseases associated with AML:
Reference:
1.Monteforte WJ, Kohnen PW. Angiomyolipomas in a case of
effects from her native kidneys. We felt that any risk
lymphangiomyomatosis syndrome: relationship to tuberous
to our patient from leaving the tumour in situ was
sclerosis. Cancer 1974; 34: 317
2. Enzinger FM, Weiss SW. Soft Tissue Tumors, 3rd edn. Mosby,
very small, and that with regular follow-up with ultra-
St. Louis USA, 1995; 405–409
sound scans any potential problems could be readily
3. Hartmann DS, Goldman SM, Freedman AC, Davis CJ,
anticipated. Whilst a large, symptomatic, or growing
Madewell JE, Sherman JL. Angiomyolipoma, ultrasonic–pathol-
mass should be explored, it is justified to leave a small,
ogic correlation. Radiology 1981; 139: 451–458
4. Colman P, Gray DWR, Dunnill M, Morris PJ. Angiomyolipoma
biopsy-proven angiomyolipoma in situ.
masquerading as malignancy in renal transplantation. Nephrol
Dial Transplant 1993; 8: 642–643
That is an interesting analysis. I like your decision-making process in accepting this kidney.
Fater 4 references, the numbering of your three references starts again !
Dear All
Very few give a focused answer
Suppose you accepted this donor (I would), what would you do to the AML?
Which kidney to take?
Suppose you accepted this donor (I would), what would you do to the AML?
Which kidney to take?
Dear Dr Ahmed,
I will recommend donating the kidney with AML (we usually keep the best kidney for the donor). And I will recommend excision of the AML before transplanting the kidney to the recipient (due to the increased risk of malignancy in the recipient secondary to immune suppression)
Reference:
1) Vicente E Torres, and York Pei. Renal angiomyolipomas (AMLs): Management. © 2023 UpToDate (accessed on 3 February 2023).
I like your analysis and approach.
Ajay
Suppose you accepted this donor (I would), what would you do to the AML?
Which kidney to take?
Thanks verey much Prof.Halawa
Reference
I like your analysis and approach. I like reflection of your own practice.
Ajay
Will use the affected kidney and remove the tumor perioperatively.
Post op: frequent surveillance
Removing peri-operatively?
Can you upload some evidence please
My final plan for such patient
1- confirm the diagnosis with MRI and refer to a uroradiology specialized
2-if the unilateral size is 4.2 cm I would refer to the transplant surgeon for excision ex-vivo if he sees that it’s accessible (the normal kidney will be kept for the donor)
3-If not accessible or bilateral donor will be rejected
I like your analysis and approach. Can you upload some evidence please
AML kidney after excision of AML
Thank you, Prof. Halawa.
First, I will do a split renal function to ascertain the kidney with the optimal better function, which will be left for the donor.
The kidney with the AML will be taken provided if ex vivo excision of the lesion is straightforward before being implanted into the recipient
Reference
I would take the kidney with AML after excision pre transplant.This will decrease risk of hemorrhage and allow recipient not to worry about the non malignant tumor for their lifetime or any other tumor related complications.
REF;
Gearge et al; Successful Utilization of a live doner kidney with angiomyolipoma.
I will do nothing to the AML.
The kidney with the AML will be harvested for donation to the recipient.
reference
Anton et al. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urol Ann. 2021
We can excise the lesion in-vivo or ex-vivo before transplantation.
In this case I will accept the donor (after excluding TSC or LAM)
(Renal AML is usually benign and a true malignant AML is rare. Risk factors for malignant AML include size >7 cm, tumor necrosis, and epithelioid carcinoma-like pattern.)
I will advise for excision of AML before transplantation in bench.
(With tumors >4 cm, the risk of vascular complications and hemorrhage is high)
I will advise to take left (AML) kidney as best kidney is to leave in donors.
Reference
Anton, Desiree Garcia; Kovvuru, Karthik; Kanduri, Swetha R; Aeddula, Narothama Reddy1; Bathini, Tarun2; Thongprayoon, Charat3; Kaewput, Wisit4; Wijarnpreecha, Karn5; Watthanasuntorn, Kanramon6; Salim, Sohail Abdul; Matemavi, Praise7; Vaitla, Pradeep; Rivera, Franco Cabeza; Cheungpasitporn, Wisit. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urology Annals 13(1):p 67-72, Jan–Mar 2021. | DOI: 10.4103/UA.UA_14_20
I would utilize the kidney with AML. Given their tendency to bleed, AMLs larger than 4cm should be excised electively since all the associated risks can be managed and reduced during ex vivo excision of the tumor.
The donor should be informed about the possible outcomes i.e., AML rupture, AML recurrence, possible graft damage.(1, 2)
References
1. Seyam RM, Alkhudair WK, Kattan SA, Alotaibi MF, Alzahrani HM, Altaweel WM. The Risks of Renal Angiomyolipoma: Reviewing the Evidence. Journal of kidney cancer and VHL. 2017;4(4):13-25. PubMed PMID: 29090118. Pubmed Central PMCID: PMC5644357. Epub 2017/11/02. eng.
2. Anton DG, Kovvuru K, Kanduri SR, Aeddula NR, Bathini T, Thongprayoon C, et al. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urology annals. 2021 Jan-Mar;13(1):67-72. PubMed PMID: 33897168. Pubmed Central PMCID: PMC8052899. Epub 2021/04/27. eng.
Should be taken the kidney with AML and excised the AML before transplant if considered.
References:
The size of AML is considered medium sized 4.6 cm in diameter (4–8 cm) characterised by having variable behaviour
However the AML lesion can be excised after donor left renal nephrectomy
Surgery or embolization may be required for active hemorrhage if occured
the patient will need regular follow up
Reference
Gopalakrishnan N, Dhanapriya J, Sakthirajan R, Dineshkumar T, Balasubramaniyan T, Haris M. Angiomyolipoma of donor kidney: Successful transplantation and 5-year follow-up. Indian J Nephrol. 2016;26(2):131-133.
Sir,
after accepting this Donor …. Should donate the kidney with AML with benchtable excision of AML with close follow up of the recipient
I will take the kidney with AML, as unilateral large (>4 cm) AML can be used if ex vivo excision of the AML appears to be straightforward
BTS/RA Living Donor Kidney Transplantation Guidelines 2018
excision of the AML before donation.
donate the kidney with AML.
I will take the kidney with AML , and to do ex vivo excision before transplantation
I would like to permit donating the kidney with AML & need to excision of the tumour
I will do a split renal function to find the kidney with the optimal better function, which will be left for the donor.
The kidney with the AML will be taken provided if ex vivo excision of the lesion is straightforward before being implanted into the recipient.
Reference
if i accepted this donor i will leave him the healthy kidney as it is usually the role and surgical removal of the mass before transplanting the kidney
I will take the affected kidney
AML can be removed extra vivo just before transplantation.
The prognosis of AML
●Small asymptomatic lesions (<4 cm) remain stable, but should be periodically evaluated,
●medium-sized lesions (4–8 cm) have variable behavior warranting serial imaging
● and large lesions(>8 cm) are associated with significant morbidity and due to the potential complications should be electively treated.
Surgery or embolization may be required for active hemorrhage
mTOR inhibitors like sirolimus and everolimus therapy were found to be associated with a reduction in AML size
Will you accept this donor?
—————————————————–
According to the guideline; bilateral AML and AML >4 cm generally preclude living kidney donation although occasionally unilateral large (>4 cm) AML can be used if ex vivo excision of the AML appears to be straightforward.
What is angiomyolipoma?
—————————————-
Classic angiomyolipoma (AML) is a triphasic, benign neoplasm composed of mature adipose tissue, smooth muscle and thick walled blood vessels .
What are the risks involved?
——————————————-
1-malignant transformation has been reported .
2-Rupture and hemorrhage.
Is this image adequate?
—————————————
Diagnosis of an AML can usually be made by imaging without recourse to biopsy; however, it is important to discriminate classic AML from the uncommon subtype of epitheloid AML, which may have a malignant phenotype.
Contrast enhanced renal CT scan, ultrasound and / or MRI are usually able to characterise whether the renal mass might be a renal cell carcinoma (RCC).
What are the other diseases associated with angiomyolipoma?
—————————————————————————–
1-Tuberous sclerosis complex (TSC).
2-sporadic lymphangioleiomyomatosis (LAM)
3- combination (TSC-LAM).
Reference ;
———————————————
1-https://fship.asnrt-edu.com/wp-content/uploads/2022/09/Guidelines-for-Living-Donor-Kidney-Transplantation.pdf.
2-BTS/RA Living Donor Kidney Transplantation Guidelines 2018.
3-Halpenny D, Snow A, McNeill G, Torreggiani WC. The radiological diagnosis and treatment of renal angiomyolipoma – current status. Clin Radiol 2010; 65: 99-108.
4-Ferry JA, Malt RA, Young RH. Renal angiomyolipoma with sarcomatous transformation and pulmonary metastases. Am J Surg Pathol 1991; 15:1083.
5-Xu XF, Hu XH, Zuo QM, et al. A scoring system based on clinical features for the prediction of sporadic renal angiomyolipoma rupture and hemorrhage. Medicine (Baltimore) 2020; 99:e20167.
Well done
Suppose you accepted this donor (I would), what would you do to the AML?
Which kidney to take?
1- Yes it can be accepted, especially if it is the only available donor
with pre-transplant excision of the tumor (in-vivo or ex-vivo)
2- Angiomyolipoma:
fat content ;pre-contrast: -20:-80 HU, homogenous, no cyst, no calcifications
if unstable: surgical exploration
3- Retroperitoneal hemorrhage
4- CT with contrast s diagnostic
5- it can be associated with Tuberous sclerosis complex (TSC)
Well done
Suppose you accepted this donor (I would), what would you do to the AML?
Which kidney to take?
References???????
we will excise the lesion in vivo (before donor nephrectomy) or ex-vivo (side bench).
Refernce:
Zahran MH, Kamal AI, Abdelfattah A, Mashaly ME, Fakhreldin I, Osman Y, Ali-El-Dein B. Outcome of Live-Donor Renal Transplants With Incidentally Diagnosed Renal Angiomyolipoma in the Donor. Transplant Proc. 2019 Jul-Aug;51(6):1773-1778.
Will you accept this donor?
Here, the immunological factors; 111 mismatch and no DSA is clearly acceptable.
Regarding angiomyolipoma, bilateral AML and AML >4 cm generally preclude living kidney donation. Sometimes unilateral large (>4 cm) AML can be used if pre-transplant excision is done.
In this case imaging of the right side is necessary to rule out bilateral lesions. Images need to be reviewed my radiologist to rule out any feature of malignancy.
What is angiomyolipoma?
Angiomyolipomas are most common tumor of kidneys composed of blood vessels, smooth muscle and adipose tissue. The majority are benign but malignant angiomyolipomas also reported.
About 0.13 to 2.2 percent of asymptomatic adults who underwent imaging having angiomyolipomas and more common among females.
It is often associated with either tuberous sclerosis complex, lymphangioleiomyomatosis, or a combination of both.
What are the risks involved?
· Hemorrhage in a large lesion.
· Malignant transformation.
Is this image adequate?
Imaging of the right side is necessary to rule out bilateral lesion.
What are the other diseases associated with angiomyolipoma?
· Tuberous sclerosis complex
· Sporadic lymphangioleiomyomatosis
· Von Hippel Lindau disease
· Neurofibromatosis type 1
Well done Eusha.
Suppose you accepted this donor (I would), what would you do to the AML?
Which kidney to take?
References???????
Will you accept this donor?
Renal angiomyolipoma (AML) is the most frequent mesenchymal tumor of the kidney.Angiomyolipoma is not an absolute contraindication to kidney donation(1). Bilateral AML and AML >4 cm generally preclude living kidney donation(2).
Although the size of this tumor is more than 4 cm, not coinciding the BTS/RA Living Donor Kidney Transplantation Guidelines 2018, I will accept this potential donor;
· 1-1-1 is a suitable mismatch and no DSA.
· Unilateral AML.
· There were reported cases of successful transplantation after donation from donors with AML with lesion greater than 4 cm [(3),(4)].
What is angiomyolipoma?
Angiomyolipoma (AML) is the most common benign neoplasm of the kidney(5). It is a tumour composed of variable amounts of mature adipose tissue, smooth muscle, and vessels derived from perivascular epitheloid cells. Its prevalence in the general population has been reported to be between 0.1 and 0.22%(6). AML appears in two distinct settings; either associated with tuberous sclerosis or sporadic in nature. The clinical presentation of AML is variable. Often patients are asymptomatic, however, they can present with the classic triad of flank pain, a tender mass and gross haematuria. They are commonly identified incidentally during radiological investigations. These tumours have the potential for life-threatening haemorrhagic rupture. In this setting patients can present with sudden acute abdominal pain and haemorrhagic shock. After renal cell carcinoma, AML is the second most common renal cause of retroperitoneal hemorrhage.
What are the risks involved?
· Malignant transformation and RCC.
· It carries risk for hemorrhage, rupture and impaired renal function.
Is this image adequate?
· CT lung to rule out Pulmonary Lymphangioleiomyomatosis (LAM) is required.
· The donor’s DNA to be examined for the presence of tuberous sclerosis.
What are the other diseases associated with angiomyolipoma?
· Tuberous Sclerosis Complex (TSC)
· Pulmonary Lymph-Angioleiomyomatosis (LAM)
· epithelioid variant of AML.
· Neurofibromatosis.
· APKD.
References
1. Anton DG, Kovvuru K, Kanduri SR, Aeddula NR, Bathini T, Thongprayoon C, Kaewput W, Wijarnpreecha K, Watthanasuntorn K, Salim SA, Matemavi P, Vaitla P, Rivera FC, Cheungpasitporn W. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urol Ann. 2021 Jan-Mar;13(1):67-72. doi: 10.4103/UA.UA_14_20. Epub 2021 Jan 19. PMID: 33897168; PMCID: PMC8052899.
2. BTS/RA Living Donor Kidney Transplantation Guidelines 2018.
3. Abboudi H, Chandak P, Kessaris N, Fronek J. A successful live donor kidney transplantation after large angiomyolipoma excision. Int J Surg Case Rep. 2012;3(12):594-6. doi: 10.1016/j.ijscr.2012.07.016. Epub 2012 Aug 21. PMID: 22982456; PMCID: PMC3484880.
4. Anton DG, Kovvuru K, Kanduri SR, Aeddula NR, Bathini T, Thongprayoon C, Kaewput W, Wijarnpreecha K, Watthanasuntorn K, Salim SA, Matemavi P, Vaitla P, Rivera FC, Cheungpasitporn W. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urol Ann. 2021 Jan-Mar;13(1):67-72. doi: 10.4103/UA.UA_14_20. Epub 2021 Jan 19. PMID: 33897168; PMCID: PMC8052899.
5. M.S. Steiner, S.M. Goldman, E.K. Fishman, F.F. Marshall The natural history of renal angiomyolipoma Journal of Urology, 150 (6) (1993), pp. 1782-1786
6. Y. Fujii, J. Ajima, K. Oka, A. Tosaka, Y.Takehara Benign renal tumors detected among healthy adults by abdominal ultrasonography European Urology, 27 (2) (1995), pp. 124-127
Well done Safi.
Suppose you accepted this donor (I would), what would you do to the AML?
Which kidney to take?
References???????
1-Will you accept this donor?
If
Bilateral AML preclude donation.
Unilateral AML, the affected kidney considered for donation.
If the AML size less than 1 cm , donte , if the size more than 4 cm the lesion must be removed pretransplantation .
2-What is angiomyolipoma?
The most typical benign tumor of the kidney is angiomyolipoma. Despite being benign, angiomyolipomas can develop to the point where kidney function is compromised or where the blood vessels enlarge and break, causing bleeding.
Angiomyolipomas are frequently found in Tuberous Sclerosis patients, who typically have several angiomyolipomas affecting both kidneys. They are frequently discovered in women who have the uncommon lung condition.
When angiomyolipomas (AML) are discovered accidentally, tuberous sclerosis complex (TSC) should be taken into account, especially in people between the ages of 18 and 40 and in those who have bilateral angiomyolipomas. AML-related hemorrhages have a 20–50% probability of occurring in people with TSC, and of those hemorrhages, 20% are life-threatening. TSC professional clinicians conduct a thorough physical examination, which may include dermatologic and ophthalmologic examinations, as well as a CT or MRI of the brain. A high-resolution CT of the lungs and pulmonary function tests are part of the LAM screening process.
Renal scans are recommended once a year for people with Tuberous Sclerosis, while patients with highly stable lesions may require less regular monitoring. There is a dearth of research in this field. Angiomyolipomas can appear at any stage of life, even if none have been discovered. Angiomyolipoma can expand quickly.
The most frequent benign kidney tumor, angiomyolipomas are either spontaneous or present in patients with Tuberous Sclerosis. 80–90% of occurrences are intermittent, and middle-aged women are the most frequently affected.
According to a longitudinal research, 80% of TSC individuals would have some kind of renal lesion by the time they are 10 years old. 17% of them are cysts, and 75% of them are angiomyolipomas. In about 60% of these kids, the angiomyolipomas grew larger. The prevalence of patients with TSC was reported to be 67 and 85%, respectively, in an autopsy study and a TSC clinic survey. The effects are same for both genders.
Angiomyolipomas that are small and those that do not contain dilated blood vessels (aneurysms) rarely cause issues, although they can grow as quickly as 4 cm in a year. Angiomyolipomas greater than 5 cm in size and those with an aneurysm are at a high risk of rupture, which is a medical emergency because it could be fatal. According to a population research, men have a cumulative risk of bleeding of 10% and women have a cumulative risk of 20%.
The development of chronic kidney disease results from the renal Angiomyolipomas occupying such a large portion of the kidney. Dialysis might be necessary if this is serious enough.
The FDA has given Everolimus approval to treat Angiomyolipomas. AMLs greater than 3 cm in diameter that are asymptomatic and growing should be treated.
Surgery is not typically necessary unless there is life-threatening bleeding in angiomyolipomas.
Due to the possibility of hemorrhage, some facilities may perform prophylactic selective embolization of the angiomyolipoma if it is more than 4 cm in diameter.
Renal scans are recommended once a year for people with Tuberous Sclerosis, while patients with highly stable lesions may require less regular monitoring. There is a dearth of research in this field. Angiomyolipomas can appear at any stage of life, even if none have been discovered. Angiomyolipoma can expand
3-What are the risks involved?
Hemorrhage , anemia, impaired kidney function ,Malignancy changes ,In female there is risk of increase in size and rupture during pregnancy .
4-Is this image adequate?
Imaging of the other side is important
MRI need to be done.
The imaging must be reviewed by a specialist uroradiologist.
5-What are the other diseases associated with angiomyolipoma?
Tuberous sclerosis multiplex
Lymphangieoleiomyomatosis
Von hippel landau disease
Neurofibromatosis type 1
Well done Asmaa.
Suppose you accepted this donor (I would), what would you do to the AML?
Which kidney to take?
References???????
The kidney with the lesion to be considered.
AML removed either in-vevo or ex-vivo.
Reference :
uptodate
3. A 46-year-old potential kidney donor came forward to donate to his brother. 111 mismatch, no DSA. CT showed angiomyolipoma in the left kidney (4.6 cm in diameter).
· Will you accept this donor? (1-4)
111 mismatch with no DSA makes him a suitable donor.
The contentious issue is the 4.6cm diameter AML lesion picked on CT scan. This is considered a medium-sized lesion with a variable course. Ideally, lesions larger than 4cm should be excised due to their increased risk of hemorrhage.
Given the associated risks i.e., risk of further growth of the tumor in the recipient and spontaneous rupture after transplantation, a decision can be made to do ex vivo or in vivo excision of the tumor. Ex vivo excision is advantageous since all the associated risks can be managed and reduced in advance. However, the donor should be informed on the possible outcomes including rupture of the AML, recurrence of the tumor and possible graft damage.
AML can occur sporadically or in association with association with tuberous sclerosis complex (TSC) and very rarely pulmonary lymphangioleiomyomatosis (LAM). A systematic review by Anton et al concluded that AML donors (without TSC or LAM) can safely donate. There was no evidence of malignant transformation of AML in the kidney transplant recipients.
This donor should be extensively investigated for suitability of organ donation, he should be assessed for underlying TSC and pulmonary LAM.
· What is angiomyolipoma? (2, 5-8)
Angiomyolipoma (AML) is a non-encapsulated kidney tumor. It is a mesenchymal tumor composed of blood vessels, smooth muscle and fat. Most AMLs are benign although malignant cases have been reported. True malignant AML is rare with risk factors for malignant AML being size >7cm, tumor necrosis and epithelioid carcinoma-like pattern. Renal AMLs have been found to be more common among females.
Renal AMLs can occur sporadically (>80%) or in association with tuberous sclerosis complex (TSC), sporadic lymphangioleiomyomatosis (LAM) or a combination (TSC-LAM). Two major histologic AML variants have been described i.e., classic variant and epithelial variant. Sporadic AMLs are usually solitary, small and slow-growing; whereas TSC-associated AMLs can be large, multiple, bilateral and tend to grow.
Most patients are asymptomatic with the tumor being detected incidentally on imaging. However, patients can present with the classic triad of a tender mass, flank pain and gross hematuria; as well as hypertension, anaemia, kidney dysfunction.
Despite its benign nature, it can be aggressive with a locoregional and venous spread, hence it is also associated with hemorrhage. Small asymptomatic lesions (<4cm) remain stable and only require periodic assessment while medium sized lesions (4-8cm) require serial imaging in view of their variable behaviour. On the other hand, large lesions (>8cm) should be managed electively since they are associated with significant morbidity and complications. The main complication being retroperitoneal hemorrhage secondary to AML rupture.
Diagnosis is usually made by imaging, preferably MRI with gadolinium contrast. When MRI is unavailable or is contraindicated, CT with and without contrast can be used.
Biopsy is not necessary unless the diagnosis is doubtful or if malignancy is suspected in which case an image guided core need le biopsy is done. Kidney biopsy is associated with increased risk of tumor rupture and hemorrhage.
Surgery (resection) or embolization is usually the gold standard management for active hemorrhage. Radiological angiographic embolization is preferred since it has the advantage of preserving renal parenchyma and avoiding the risks associated with surgery. Surgical exploration can also be done in cases of suspected malignancy or in cases where a percutaneous needle biopsy is not feasible or is unsuccessful in providing a diagnosis.
Routine surveillance is usually done for patients with sporadic AMLs at low risk for malignant transformation. mTORi have been found beneficial in patients with multiple rapidly growing (>2.5mm per year).
· What are the risks involved?
– Spontaneous rupture after transplantation. Factors associated with an increased risk of hemorrhage include –
o AML size (>6cm),
o increased vascularity,
o intralesional aneurysms (>5mm at greatest width),
o exophytic growth pattern
o pregnant females
o uncontrolled hypertension
o anticoagulation/ coagulopathy
– Malignant transformation – rapid increase in tumor size/ further growth of the tumor in the recipient especially “fat-poor” and “fat-invisible” AML
· Is this image adequate? (9)
– Unenhanced CT abdomen is the commonly used diagnostic modality but it cannot clearly do fat quantification.
– MRI abdomen is very sensitive in diagnosing and differentiating fat-poor AML lesions from renal malignancies. Renal AML can be classified as fat-rich, fat-poor and fat-invisible based on fat quantification as seen on MRI.
· What are the other diseases associated with angiomyolipoma?
– Tuberous sclerosis complex (TSC)
– Lymphangioleiomyomatosis (LAM)
References
1. Anton DG, Kovvuru K, Kanduri SR, Aeddula NR, Bathini T, Thongprayoon C, et al. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urology annals. 2021 Jan-Mar;13(1):67-72. PubMed PMID: 33897168. Pubmed Central PMCID: PMC8052899. Epub 2021/04/27. eng.
2. Gopalakrishnan N, Dhanapriya J, Sakthirajan R, Dineshkumar T, Balasubramaniyan T, Haris M. Angiomyolipoma of donor kidney: Successful transplantation and 5-year follow-up. Indian journal of nephrology. 2016 Mar-Apr;26(2):131-3. PubMed PMID: 27051138. Pubmed Central PMCID: PMC4795429. Epub 2016/04/07. eng.
3. Yamakado K, Tanaka N, Nakagawa T, Kobayashi S, Yanagawa M, Takeda K. Renal angiomyolipoma: relationships between tumor size, aneurysm formation, and rupture. Radiology. 2002 Oct;225(1):78-82. PubMed PMID: 12354988. Epub 2002/10/02. eng.
4. Seyam RM, Alkhudair WK, Kattan SA, Alotaibi MF, Alzahrani HM, Altaweel WM. The Risks of Renal Angiomyolipoma: Reviewing the Evidence. Journal of kidney cancer and VHL. 2017;4(4):13-25. PubMed PMID: 29090118. Pubmed Central PMCID: PMC5644357. Epub 2017/11/02. eng.
5. Vos N, Oyen R. Renal Angiomyolipoma: The Good, the Bad, and the Ugly. Journal of the Belgian Society of Radiology. 2018 Apr 20;102(1):41. PubMed PMID: 30039053. Pubmed Central PMCID: PMC6032655. Epub 2018/07/25. eng.
6. Çalışkan S, Gümrükçü G, Özsoy E, Topaktas R, Öztürk Mİ. Renal angiomyolipoma. Revista da Associação Médica Brasileira. 2019;65:977-81.
7. Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, et al. Update on the Diagnosis and Management of Renal Angiomyolipoma. The Journal of urology. 2016 Apr;195(4 Pt 1):834-46. PubMed PMID: 26612197. Epub 2015/11/28. eng.
8. Wang C, Li X, Peng L, Gou X, Fan J. An update on recent developments in rupture of renal angiomyolipoma. Medicine. 2018 Apr;97(16):e0497. PubMed PMID: 29668633. Pubmed Central PMCID: PMC5916643. Epub 2018/04/19. eng.
9. Song S, Park BK, Park JJ. New radiologic classification of renal angiomyolipomas. European journal of radiology. 2016 Oct;85(10):1835-42. PubMed PMID: 27666625. Epub 2016/09/27. eng.
UpToDate
This is an excellent answer with references. Very impressed Dr Kibe.
Suppose you accepted this donor (I would), what would you do to the AML?
Which kidney to take?
I would utilize the kidney with AML. Given their tendency to bleed, AMLs larger than 4cm should be excised electively since all the associated risks can be managed and reduced during ex vivo excision of the tumor.
The donor should be informed about the possible outcomes i.e., AML rupture, AML recurrence, possible graft damage. (1,2).
References
1. Anton DG, Kovvuru K, Kanduri SR, Aeddula NR, Bathini T, Thongprayoon C, et al. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urology annals. 2021 Jan-Mar;13(1):67-72. PubMed PMID: 33897168. Pubmed Central PMCID: PMC8052899. Epub 2021/04/27. eng.
2. Seyam RM, Alkhudair WK, Kattan SA, Alotaibi MF, Alzahrani HM, Altaweel WM. The Risks of Renal Angiomyolipoma: Reviewing the Evidence. Journal of kidney cancer and VHL. 2017;4(4):13-25. PubMed PMID: 29090118. Pubmed Central PMCID: PMC5644357. Epub 2017/11/02. eng.
Will you accept this donor?Further workup is needed before decision can be made; imaging of the right side to rule out bilateral lesions. Images need to be reviewed my uroradiologist to rule out feature of malignancy (intratumoral necrosis, intratumoral calcifications, presence of lymphadenopathy, or metastasis).
Bilateral AML and AML >4 cm generally preclude living kidney donation although occasionally unilateral large (>4 cm) AML can be used if ex vivo excision appears to be straightforward.
What is angiomyolipoma?Angiomyolipomas are neoplasms occurring in the kidney composed of varying amounts of tissues resembling blood vessels, smooth muscle, and adipose tissue. The majority are benign but malignant AMLs have been reported.
AMLs have been reported in 0.13 to 2.2 percent of asymptomatic adults who underwent imaging. It is to be more common among females perhaps related to the effect of oestrogen on the growth of the lesion.
It occurs sporadically or in association with either tuberous sclerosis complex, sporadic lymphangioleiomyomatosis, or a combination of both.
There are two major histological variants of AMLs: classic and epithelioid. In addition, there is a rare cystic variant, called angiomyolipoma with epithelial cysts. Variants are distinguished from each other only by biopsy.
Clasiic varients re benign but can be locally invasive, extending into the perirenal fat or, rarely, the collecting system, renal vein, or inferior vena cava and right atrium.
The epithelioid variant can undergo malignant transformation.
Angiomyolipoma with epitheliod cyst is a benign lesion with no evidence of metastasis or recurrence following surgical excision
Most renal AMLs are asymptomatic. AMLs usually come to medical attention as an incidental finding on imaging performed for a different indication.
Among patients who are symptomatic, features related to haemorrhage appear to be most common.These include abdominal or flank pain and tenderness, haematuria, hypertension, anaemia, and kidney function impairment.
The diagnosis of renal AML is usually made by imaging. Preferably gadolinium enhanced MRI. CT with or without contrast is an alternative if MRI is not available.
A biopsy of the lesions is usually not necessary, unless the diagnosis is not clear upon imaging or if malignancy is suspected.
What are the risks involved?Tumors > 4cm carry the risk of vascular complications and retroperitoneal haemorrhage.
Epithelioid AMLs can undergo malignant transformation, distant metastasise and recur locally after removal.
Is this image adequate?Imaging of the right side is needed to rule out bilateral lesions.
What are the other diseases associated with angiomyolipoma?Tuberous sclerosis complex, sporadic lymphangioleiomyomatosis or a combination of both. Von Hippel Lindau disease.
Well done.
Will you accept this patient
This patient has unilateral AML with 4.5 cm ,in this case we need to see if it is operable then we can do partial nephrectomy and then proceed with transplant with marginal kidney chriteria .
AML is the most common benign tumures whic is present in the kidney ,with genetic background .
AML can be part of the SRMs of indeterminate significance represent a heterogeneous group of lesions that range from benign masses and cysts to indolent and aggressive malignancies. SRMs and complex cystic masses (typically with a solid enhancing component) are more likely to be malignant and warrant further evaluation.
SRMs can be benign kidney lesions, and benign lesions are more common in females and with smaller tumors. In a single-institution experience, 311 of 2675 surgically resected tumors were benign [21]. These included oncocytoma (75 percent), angiomyolipoma (11 percent), and metanephric adenoma (3 percent).
Angiomyolipomas can be reliably distinguished on imaging as an enhancing mass that contains macroscopic fat and no calcifications
When renal angiomyolipomas are bilateral, patients have an 80 to 90 percent chance of having tuberous sclerosis. Patients with tuberous sclerosis also have an increased frequency of RCC, as approximately 1 to 2 percent will develop this malignancy; hence, surveillance for RCC should be considered in this population and they are not fit for donation .
AML of less than 4cm and unilateral can proceed for donation with serial follow up and close observation.Angiomyolipomas require further management because of the risk for hemorrhage, which depends partly on their size and growth rate .
I thing CT abdomen is adequate to evaluate the AML and no need for further test .
Other disease which associated with this AML includes:
1..Neurofibromatosis type 1.
2.Tuberous sclerosis complex.
3.Von Hippel Lindau disease.
4.Lymphangioleiyomyomatosis.
references
uptodate
CT,MRIare useful to examine the lungs, assess the fat content and rule out distant spread.
WILL YOU ACCEPT THE DONOR?
Yes, subject to the following;
Ref- British Transplantation Society guidelines – 2018;
WHAT IS AN ANGIOMYOLIPOMA?
This is the commonest benign tumors of the kidney caused by genetic mutation of TSC1 or TSC2 and affects 0.2-0.6% of people especially women over 50 years of age. The ones that turn hemorrhagic are life threatening.
RISKS INVOLVED;
Increased risk of bleeding in;
1. Females on hormone based contraceptives.
2.Pregnancy.
3. Large sized AML esp more than 4 cm.
ADEQUACY OF IMAGING;
Bilateral imaging of both kidneys with either a CT SCAN or MRI needed for proper evaluation pre transplant.
ASSOCIATIONS WITH AML.
1.Neurofibromatosis type 1.
2.Tuberous sclerosis complex.
3.Von Hippel Lindau disease.
4.Lymphangioleiyomyomatosis.
REFERENCES.
Uptodate – Renal AML;Dec 2022.
Andrews PA et al; British Transplantation Society/Renal Association UK guidelines for living donor kidney transplantation 2018;summary of updated guidelines of transplantation 2018;July;102(7)e307.
please revise the 4th recommendation of BTS
What is your decision in this case.?
Bilateral AML and AML > 4cm generally preclude living kidney donation although occasionally unilateral large (>4cm) AML can be used if ex vivo excision of the AML appears to be straight forward.
I will proceed with donation.
Thanks Prof.
Will you accept this donor?
o For living kidney donors, bilateral Angiomyolipoma (AML) preclude donation.
o In unilateral disease, generally only the affected kidney should be considered for donation
o If the AML is <1 cm, the affected kidney may be considered for donation, or in a male donor can be left in situ in the donor’s remaining kidney.
o If an AML is 4 cm or larger, donation should only be contemplated if excision of the AML is possible, because of the risk of subsequent symptoms.
What is Angiomyolipoma?
Angiomyolipoma (AML) is a triphasic, benign neoplasm composed of mature adipose tissue, smooth muscle and thick walled blood vessels and can occur as an incidental finding in donor work-up
What are the risks involved?
Hemorrhage
Malignancy changes
In female there is risk of rupture during pregnancy
Is this image adequate?:
o We need to do MRI
o The imaging must be reviewed by a specialist uroradiologist.
What are the other diseases associated with angiomyolipoma?:
1- Tuberous sclerosis
2- Lymphangieoleiomyomatosis
3- Von hippel landau disease
4- Neurofibromatosis type 1
References :
1-Nalesnik MA, Woodle ES, Dimaio JM, et al. Donor-transmitted malignancies in
organ transplantation: assessment of clinical risk. Am J Transplant 2011; 11:
1140-7.
2-Zhang S, Yuan J, Li W, Ye Q. Organ transplantation from donors (cadaveric or
living) with a history of malignancy: review of the literature. Transplant Rev
(Orlando) 2014; 28: 169-75.
1-Will you accept this donor?
This not optimal for Living donation and if accepted will underwent surgical excision before transplantation and according Tx Surgeon
Angiomyolipoma bilateral precludes from donation
2-What is angiomyolipoma?
Angiomyolipomas (AMLs) are neoplasms occurring in the kidney that are composed of varying amounts of tissues resembling blood vessels, smooth muscle, and adipose tissue. The vast majority of AMLs are benign but malignant AMLs have been reported.
3-What are the risks involved?
-Hemorrhage appear to be most common. These include abdominal or flank pain (associated with mass effect of the hemorrhage) and tenderness, hematuria, hypertension, anemia, and kidney function impairment.
-Hemorrhage is the most feared clinical complication of AMLs due to its severe nature, often resulting in significant pain, anemia, and sometimes, hemorrhagic shock.
-Renal AMLs are the most common cause of Wunderlich syndrome, a life-threatening, nontraumatic kidney hemorrhage into the subcapsular and perirenal spaces.
4-Is this image adequate?
Not adequate ;.
For further radiology;.CT or MRI, then we obtain a needle-guided biopsy of the kidney lesion.
5-What are the other diseases associated with angiomyolipoma?
-Tuberous sclerosis complex (TSC);
-Sporadic lymphangioleiomyomatosis (LAM);.
-Combination of LAM and TSC (TSC-LAM) likely account for a small minority of cases.
References;.
UP TO DATE
Well done.
CT showed a mass in the left kidney for DD. Diagnosis should be made before accepting or rejecting the patient. it can be a benign tumor-like angiomyolipoma or malignant like RRC. A uroradiology specialist is needed to confirm the diagnosis and usually, angiomyolipoma is a fat-rich lesion that can be diagnosed by MRI but sometimes a biopsy is needed.
Once angiomyolipoma is diagnosed size, site, number, and associations need to be evaluated as per BTS 2018 guidelines as
Angiomyolipoma bilateral precludes from donation
Soliteray angiomyolipoma with a Size of more than 4cm should be accessible for surgical removal before transplantation
Solitary angiomyolipoma with size from 1cm-4cm can be directly transplanted with serial ultrasound follow-up post-transplant or ex-vivo excision pretransplant
Solitary angiomyolipoma with a size of less than 1cm can be transplanted directly and no need for surgical removal.
· Tuberous sclerosis and lymphoangiomyomatosis LAM are associated with kidney angiomyolipoma and preclude form donation. What is angiomyolipoma?
Risk of hemorrhage especially if large in size by more than 4cm or increasing in size by more than 2.5cm per year
Risk of malignant transformation which is very rare
Risk of ESRD which occurs in TS when there are multiple lesions
As mentioned before MRI confirmation is needed, also evaluation of other kidneys and exclusion of TS and LAM
TS and LAM
My final plan for such patient
1- confirm the diagnosis with MRI and refer to a uroradiology specialized
2-if the unilateral size is 4.2 cm I would refer to the transplant surgeon for excision ex-vivo if he sees that it’s accessible or bilateral we will proceed if not donor will be rejected
REFERENCES
Well done.
-Will you accept this donor?
The size of AML is considered medium sized 4.6 cm in diameter (4–8 cm) characterised by having variable behaviour requiringing serial imaging
Sirolimus and everolimus therapy has favourable effect in reducing AML size ,
This donor has 111 mismatch and no DSA
So in view of shortage of available donors if no other available options this donor can be accepted with donor and recipient counselling
What is angiomyolipoma?
Angiomyolipomas of the kidney are benign neoplasms non-encapsulated consist of thickened vessels , smooth muscle and adipose tissue originating from the mesenchymal elements of the kidney .
In sporadic cases it’s incidence can reach 1-3% and sporadic AML tumor is usually large, single, and unilateral with right kidney commonly involved but tumors greater than 4 cm are symptomatizing.
In tuberous sclerosis cases it’s incidence ranges from 45-80% .
Not many reported renal donors with angiomyolipomas.
What are the risks involved?
Haemorrhage specially in large tumors due to abnormal vessels and pseudoaneurysm.
Retroperitoneal bleeding that can be fatal ‘’Wunderlich syndrome”.
Rarely sarcomatous changes with spreading to surrounding tissues.
Very small percentage can be cancerinogenic.
Is this image adequate?
CT multidetector ,MRI .
What are the other diseases associated with angiomyolipoma?
Reference
-Gopalakrishnan N, Dhanapriya J, Sakthirajan R, Dineshkumar T, Balasubramaniyan T, Haris M. Angiomyolipoma of donor kidney: Successful transplantation and 5-year follow-up. Indian J Nephrol. 2016;26(2):131-133.
Well done
Will you accept this donor?
If the angiomyolipoma is small sized sporadic and after exclusion of associated syndromes, he can be accepted as the potential kidney donor as in the current scenario. sirolimus and everolimus therapy was found to be beneficial in reducing AML size in nontransplant cases. Angiomyolipoma size in our index case is 4.6 cm which considered medium-sized lesions (4–8 cm) have different behaviour need serial follow up imaging.
What is angiomyolipoma?
It is a non-encapsulated benign neoplasm composed of thickened vessels, smooth muscle and adipose tissue that arise from the mesenchymal elements of the kidney. They occur as sporadic, isolated variant in 80% of cases.
Associated syndromes: around 20%
Associated with tuberosclerosis complex (TSC) or pulmonary lymphangioleiomyomatosis.
Tuberous sclerosis: In TSC, AMLs are usually small, multiple, bilateral with equal sex distribution, and their prevalence is age related.
The higher frequency in females is due to increased expression of estrogen and progesterone receptors in these tumors. Most of angiomyolipomas are benign and asymptomatic. Symptoms develop when tumor size reaches 4 cm or more. Hemorrhage is the major complication of these highly vascular tumors due to the presence of abnormal vessels and pseudoaneurysms, especially in large angiomyolipomas (>8 cm). Rare complication is sarcomatous changes with infiltration into surrounding tissues.
Is this image adequate?
Imaging with CT or MRI, surveillance is typically performed with the same modality at 6 and 12 months and then yearly for five years. Thereafter, if the lesion size is stable, kidney ultrasound can be performed every three years.
References:
Yamakado K, Tanaka N, Nakagawa T, Kobayashi S, Yanagawa M, Takeda K. Renal angiomyolipoma: Relationships between tumor size, aneurysm formation, and rupture. Radiology. 2002;225:78–82
Well done. but mTOR inhibitors are for TSC2 which is more prone to complications.
AML is a benign neoplasm with an incidence of 45 to 80% of patients with TSC.
1 to 3%in sporadic cases and it composed of adipose tissue, smooth muscle, and thick-walled blood vessels and risk of rupture and hemorrhage due to intralesional aneurysm
1. Weerakkody Y, Amini B. “Renal angiomyolipoma”. Radiopaedia. Retrieved 2019-08-02.
2. ^ Jump up to:a b c d e f g h i j k l m Bissler JJ, Henske EP. Renal Manifestations of Tuberous Sclerosis Complex. In: Kwiatkowski DJ, Wiittlemore DJ, Thiele EA, editors. Tuberous Sclerosis Complex: Genes, Clinical Features and Therapeutics. Wiley-VCH Verlag GmbH; 2010. p. 321–325. ISBN 3-527-32201-9.
3. Badruddoza SM, Jahan RA. Renal angiomyolipoma. Saudi J Kidney Dis Transpl. 2012;23:358–60. [PubMed] [Google Scholar]
Thankyou but if the decision is to leave it then you can decide for SAE.
But if you take it for donation the surgeon can deal with it after nephrectomy then implant it.
Anyway the surgeons here are the decision takers.
Will you accept this donor?
This is an axial view of CT Renal- Unenhanced and contrast image. It is showing a lesion in the kidney which does not enhance on contrast injection. It has been reported as 4.6 cm Angiomyolipoma- AML. It will be sensible to see coronal and axial images and assess the opposite kidney.
Kidney with AML can be considered for donation if Ex vivo excision is possible. Kidney with AML < 1 cm can be transplanted without excision. Those with size > 1 cm need ex vivo excision before transplantation. However this case has to be discussed at Transplant MDT and patient and family have to be counselled in detail before transplantation.
What is angiomyolipoma?
AML are benign renal tumours and composed of blood vessels, smooth muscles and fat. AML . Term first coined in 1951 by Morgan. 80% are sporadic and 20% are associated with tuberous sclerosis ( TS). Thought to be derived from perivascular epithelioid cells. It may be hormone (oestrogen) dependent. It is more common in females. Extremely rare before puberty. Accelerated growth in pregnancy with increased risk of rupture. Rapid growth associated with OCP usage.
Non renal AML can be found in lymph nodes, renal vein and retroperitoneum
What are the risks involved?
The main risk is spontaneous bleeding into retroperitoneal space– Wunderlich syndrome.
Large AML can lead to renal tissue loss and higher risk of CKD. Risk of bleeding is high in pregnancy and with oral contraceptive use.
Is this image adequate?
CT renal dynamic and MR with contrast can be used to distinguish between benign and malignant renal lesions. Presence of fat (-20 HU) within the lesion on CT virtually excludes Renal cell carcinoma.
What are the other diseases associated with angiomyolipoma?
AML are associated with TS. TS is an Autosomal dominant condition characterised by mental retardation , epilepsy (fits), adenoma sebaceum (facial angiofibromas – zits), and ash-leaf patches.
AML can be associated lymphangiolieymatosis and von Hippel-Lindau syndrome
References
1- Versluis J, Pandey M, Flamand Y,et al. Prediction of life-threatening and disabling bleeding in patients with AML receiving intensive induction chemotherapy. Blood Adv. 2022 May 10;6(9):2835-2846.
2- BAUS FRCS Urol revision notes
Well done.
Will you accept this donor?
Yes, I will.
It is a case of angiomyolipoma with good matching related kidney donor with no DSA.
Will accept unilateral large (>4 cm) AML if ex vivo excision of the AML appears to be straightforward but if bilateral AML and AML >4 cm generally preclude living kidney donation.
A kidney with an AML <1 cm may be considered for donation or left in situ in the donor’s remaining kidney.
Kidneys containing a single AML between 1 and 4 cm can be considered for donation depending on its position, consideration of whether ex vivo excision of the AML is straightforward (1).
What is angiomyolipoma?
Classic angiomyolipoma (AML) is a tri-phasic, benign neoplasm composed of mature adipose tissue, smooth muscle and thick walled blood vessels (2).
What are the risks involved?
The main risk is Increasing vascularity or an intra lesional aneurysm that is 5 mm or larger are also associated with an increased risk of hemorrhage (3).
Risk of bleeding is so much increased with pregnancy due to rapid growth of AML tissue which is mainly due to hormonal changes of pregnancy and increasing of COP(4).
Also growing of AML will decrease renal tissue with progressing to CKD.
Is this image adequate?
Contrast enhanced renal CT scan, ultrasound and / or MRI can usually
distinguish between benign lesions such as angiomyolipoma (AML) or
malignancy such as renal cell carcinoma (RCC). Review by a specialist
uroradiologist is recommended(1).
Magnetic resonance imaging (MRI) with gadolinium contrast is preferred but Computed tomography (CT) without and with contrast enhancement is reasonable when an MRI is not available or is inappropriate.
What are the other diseases associated with angiomyolipoma?
Tuberous sclerosis complex (TSC), sporadic lymphangioleiomyomatosis (LAM), or a combination.
References:
1-BTS/RA Living Donor Kidney Transplantation Guidelines 2018.
2- Halpenny D, Snow A, McNeill G, Torreggiani WC. The radiological diagnosis and treatment of renal angiomyolipoma – current status. Clin Radiol 2010; 65: 99-108.
3- Xu XF, Hu XH, Zuo QM, Zhang J, Xu HY, Zhang Y. A scoring system based on clinical features for the prediction of sporadic renal angiomyolipoma rupture and hemorrhage. Medicine (Baltimore). 2020;99(20):e20167. doi:10.1097/MD.0000000000020167.
4- Lewis EL, Palmer JM. Renal angiomyolipoma and massive retroperitoneal hemorrhage during pregnancy. West J Med. 1985;143(5):675-676.
Thank the privellage of MRI is the estimation of fat content.
Large renal mass consistent with AML is seen in the supplied picture.
Angiomyolipoma Kidney AML is a common benign mesenchymal tumor that poses no threat to health and shows no signs of progressing to malignancy in the vast majority of instances. But some histological subtypes, especially epithelioid ones, can develop into cancer. Donor kidneys with AML should be removed since the disease can induce retroperitoneal bleeding.
When a donor has a significant lesion on one side (>4 cm), the healthy kidney is the one that gets donated. Kidney donation and lesion removal are standard treatments for patients with unilateral small AML.
In the settings of bilateral disease, underlying genetic disease should be suspected and kidney should be discarded for donation.
What potential risks exist?
Women who are pregnant or have a history of tuberous sclerosis have a higher chance of rupture from AML, which can cause hypovolemic shock, severe discomfort, and possibly a malignant form of the disease.
Does the picture adequately represents the situation?
Both kidneys should be seen on this imaging modality, as including both kidneys requires a shift in strategy and may halt donation.
In addition to angiomyolipoma, what other disorders are there?
Differential diagnosis includes the following: Tuberous sclerosis, lymphangioleiomyomatosis, Neurofibromatosis,Von Hippel-Lindau (VHL) disorder
YOU will donate the healthy kidney in a cadaveric donor.!!
Donor HLA 3/6 mismatch and no DSA with incidental finding of AML diameter 4.6cm.
we need to confirm that the donor doesn’t have tuberous sclerosis or pulmonary lymphangioleiomyomatosis or the AML is not the epithelial variant which are associated with malignancy.
If this is a sporadic AML then we can accept this donor having a good match
and AML confirmed to be unilateral and ex vivo excision is feasible.
The potential donor and recipient should be aware about the condition the potential risk associated. Case should be evaluated by the urologist and transplant surgeon.
Renal AML is the most frequent mesenchymal tumor of the kidney.
– It is a heterogeneous tumor with varying elements of smooth muscle, adipose tissue, and vascular elements in different ratios.
– There are two major histologic variants:
– Epithelioid AMLs can undergo malignant transformation, distant metastasis, and can be associated with local recurrence after removal.
It can occur:
– AML is asymptomatic and could be detected incidentally on imaging in 80 %.
AML has increased risk of rupture leading to hypovolemic shock, sever pain, sometimes malignant form, especially in those associated with tuberous sclerosis and in pregnant females (2).
Ref;
Well done.
The index renal donor has 11 with no DSA, but on evaluation, is detected to have a renal mass- angiomyolipoma (AML) which is 4.6 cm in diameter.
As per the BTS guidelines, the donation can be contemplated if the mass unilateral, and is resectable (1).
Bilateral AML preclude donation. Kidney with unilateral AML, if less than 1 cm, can be transplanted with regular sonological follow-up post-transplant. Non-involved kidney can be transplanted if the donor is male, but in case of female donors, the affected kidney should be transplanted (1).
2. What is angiomyolipoma?
Angiomyolipoma is a benign neoplasm, composed of thick-walled blood vessels, smooth muscle cells, and mature adipose tissue (1). These arise from mesenchymal tissue of the kidney.
3. What are the risks involved?
AML has increased risk of rupture, pain, hypovolemic shock, malignant form, especially in those associated with tuberous sclerosis and in pregnant females (2).
4. Is this image adequate?
No. We need imaging of both the kidneys. Bilateral involvement precludes donation.
5. What are the other diseases associated with angiomyolipoma?
Diseases associated with angiomyolipoma include tuberous sclerosis complex, neurofibromatosis type 1, Von-Hipple Lindau disease, and lymphangioleiomyomatosis (3).
References:
1. British Transplantation Society. Renal Association Guidelines for Living Donor Kidney Transplantation, 4th ed.; British Transplantation Society: Macclesfield, UK, 2018; Available online: https//bts.org.uk/wp-content/uploads/2018/07/FINAL_LDKT-guidelines_June-2018.pdf (accessed on 30 January 2023).
2. Anton DG, Kovvuru K, Kanduri SR, Aeddula NR, Bathini T, Thongprayoon C, Kaewput W, Wijarnpreecha K, Watthanasuntorn K, Salim SA, Matemavi P, Vaitla P, Rivera FC, Cheungpasitporn W. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urol Ann. 2021 Jan-Mar;13(1):67-72. doi: 10.4103/UA.UA_14_20. Epub 2021 Jan 19. PMID: 33897168; PMCID: PMC8052899.
3. Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, Perry KT, Nadler RB. Update on the Diagnosis and Management of Renal Angiomyolipoma. J Urol. 2016 Apr;195(4 Pt 1):834-46. doi: 10.1016/j.juro.2015.07.126. Epub 2015 Nov 21. PMID: 26612197.
In the case you choose the non affected kidney please perform a split function to leave the better one.
Thank you Professor.
If the size is 4.6 cm, can it be kept with the donor even if the affected kidney has better function?
My understanding is that kidney with this size AML should be taken out and transplanted after resection of the AML.
It is left for your judgement :
if the affected one has a near function take it and deal with the tumour.
Exactly in the index case with this size you should take it and deal with the tumour on the bench (as easier control of bleeding ) then transplant it offcoarse this is left to the surgoen to decide.
Thank you Professor
The above patient has a very good immunological matching, however, accepting or rejecting this patient will depend on the following conditions:
Accept if:
Reject if
What is angiomyolipoma
AML is a neoplasm that occurs in the kidney and it is usually made up of a composition of blood vessels, smooth muscles, and adipose tissue. Most AMLs are benign in nature with very few that have been reported to be malignant i.e epithelioid AML. Also, the majority of them are found incidentally during a radiological investigation.
The incidence is reported to be 0.13- 2.2% with female predominance as estrogen has been implicated, The majority are said to be sporadic and the rest has an association with other disease entity.
Variants of AML
Clinical presentation
Diagnosis
Risk involved
Hemorrhage from ruptured blood vessels or aneurysm
Malignant transformation
Is this image adequate
Other diseases associated with AML
References
Well done.
1-Will you accept this donor? NO
SIZE MORE THAN 4 CM MORE LIKELY FOR BLEEDING
INSPITE OF THIS THERE IS ANOTHER OPINION
there is a rare possibility of malignant transformation of AML
The safety of donors with AML and their kidney transplant recipients has not been well established.
ACCORDING THIS STUDY
Fourteen studies with a total of 16 donors with AML were identified.
None of the donors had a diagnosis of tuberous sclerosis complex (TSC), pulmonary lymphangioleiomyomatosis (LAM), or epithelioid variant of AML.
Donor age ranged from 35 to 77 years, and recipient age ranged from 27 to 62 years
.
Ninety-two percent of the donors were female.
Only 8% were deceased donor renal transplant
The majority underwent ex vivo resection (65%) before transplantation, followed by no resection (18%), and the remaining had in vivo resection.
Tumor size varied from 0.4 cm to 7 cm, and the majority (87%) were localized in the right kidney.
Follow-up time ranged from 1 to 107 months.
Donor creatinine prenephrectomy ranged 0.89-1.1 mg/dL and postnephrectomy creatinine 1.0-1.17 mg/dL
. In those who did not have resection of the AML, tumor size remained stable. None of the donors with AML had end-stage renal disease or died at last follow-up.
None of the recipients had malignant transformation of AML.
Conclusion: These findings are reassuring for the safety of donors with AML (without TSC or LAM) as well as their recipients without evidence of malignant transformation of AML. As such, this can also positively impact the donor pool by increasing the number of available kidneys.
Urol Ann2021 Jan-Mar;13(1):67-72. doi: 10.4103/UA.UA_14_20. Epub 2021 Jan 19
2-What is angiomyolipoma?
are benign neoplasms occurring in the kidney and are composed of varying amounts of tissue resembling blood vessels, smooth muscles, and adipose. The vast majority of AMLs are benign but malignant transformation has been reported
3-What are the risks involved?
*** Bleeding from renal AMLs can be mild or catastrophic resulting in hemorrhagic shock, loss of function of the affected kidney, and death Patients with AML who develop active bleeding should receive resuscitative measures (if hemodynamically unstable) and, if feasible, undergo prompt angiography and selective artery embolization (SAE) to stop the bleeding.
**** risk for malignant transformation —
The two major histologic variants of AMLs are classic and epithelioid.
Classic variants, not undergo malignant transformation, are “fat-rich” by imaging.
epithelioid variants, undergo malignant transformation, are “fat-poor” or “fat-invisible” by imaging.
Thus, patients with a sporadic AML that is “fat-poor” or “fat-invisible” should generally undergo percutaneous core needle biopsy However, if imaging reveals intratumoral necrosis, intratumoral calcifications, presence of lymphadenopathy, or suspected metastasis, then a biopsy is not necessary and the lesion should be surgically excised
Biopsy findings that are consistent with a high risk of malignant transformation include: >70 percent epithelioid cells; vascular invasion; ≥2 mitotic figures per 10 high-power fields; atypical mitotic figures; and necrosis
4-Is this image adequate?
YES IT IS
5-What are the other diseases associated with ?
tuberous sclerosis complex (TSC), sporadic lymphangioleiomyomatosis (LAM)
.References
Urol Ann2021 Jan-Mar;13(1):67-72. doi: 10.4103/UA.UA_14_20. Epub 2021 Jan 19
YOU need to exclude bilaterality,CT to exclude lung and other site affection,MRI for fat evaluation.
Will you accept this donor?
-Any potential donors with an incidental renal mass must have this diagnosed and managed carefully before considering donation.
– There has been a growing literature with case reports of successful renal transplantation after excision of AML.
– Evaluate all patients with newly diagnosed AML for possible TSC, LAM.
– Patients with multiple (three or more), bilateral, or larger (ie, ≥4 cm) AMLs are more likely to have TSC.
BTS/RA guideline 2018 stated:
– Bilateral AML and AML >4 cm generally preclude donation
– Unilateral large (>4 cm) AML can be used if ex vivo excision of the AML appears to be straightforward with subsequent donation
– Unilateral solitary AML <4 cm with typical CT not usually preclude donation.
– AML <1 cm may be considered for donation or left in situ in the donor’s remaining kidney.
– Single AML between 1-4 cm can be considered for donation, with consideration for AML ex vivo excision or whether it can be left in situ in the recipient and followed with serial ultrasound imaging. (C1)
The potential donor will be accepted if:
– AML confirmed to be unilateral and ex vivo excision is feasible.
– Exclude the risk that might increase malignant transformation that associated with: tuberous sclerosis (TS) or lung LAM, Epithelioid type.
-The potential donor and recipient should be aware about the condition the potential risk associated.
-Case should be evaluated by the urologist and transplant surgeon.
What is angiomyolipoma?
– Renal AML is the most frequent mesenchymal tumor of the kidney
– It is a heterogeneous, triphasic tumor with varying elements of smooth muscle, adipose tissue, and vascular elements.
– There are two major histologic variants: classic and epithelioid. In addition, there is a rare cystic variant, called angiomyolipoma with epithelial cysts.
– Epithelioid AMLs can undergo malignant transformation, distant metastasis, and can be associated with local recurrence after removal.
– It can occurs:
– Females more commonly affected than males
– AML is commonly asymptomatic and could be detected incidentally on imaging in 80 %.
– Clinical manifestation is related to AML size.
What are the risks involved?
Most of these risks are infrequent in sporadic form.
– Left flank pain, tenderness, hematuria, and fatigue when the size > 4 cm.
– Potential vascular aneurysm.
– Compression of renal parenchyma contributes to CKD with worsening renal failure
– Urinary concentration defects
– Essential hypertension
– Hemorrhage at the presentation (Wunderlich syndrome) in < 15% , life-threatening, nontraumatic kidney hemorrhage into the subcapsular and perirenal space, needing immediate intervention whereas < 10% of them appear with hypovolemic shock.
– True malignant transformation is rare.
Is this image adequate?
o This image is not adequate as it showed only left kidney, full evaluation is mandatory.
o Imaging plays a central role in the diagnosis and management of renal AML
o The fundamental diagnostic criterion of classic angiomyolipoma is detecting a large amount of adipose tissue on radiological imaging.
o Renal AML is classified as fat‑rich, fat‑poor, and fat‑invisible AMLs.
o Fat poor and fat invisible are not clearly differentiated by abdominal CT, and this represents a diagnostic challenge that can often be resolved using MRI to visualize the mass
o MRI abdomen is very sensitive in diagnosing and distinguishing fat‑poor AML lesions from renal malignancies RCC.
o Renal biopsy can provide an accurate diagnosis of AML, however, it is rarely performed considering the risk of tumor rupture and hemorrhage
What are the other diseases associated with angiomyolipoma?
– Tuberous sclerosis.
– Lymphangioleiomyomatosis LAM.
References:
o Anton DG, Kovvuru K, Kanduri SR, Aeddula NR, Bathini T, Thongprayoon C, Kaewput W, Wijarnpreecha K, Watthanasuntorn K, Salim SA, Matemavi P, Vaitla P, Rivera FC, Cheungpasitporn W. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urol Ann. 2021 Jan-Mar;13(1):67-72. doi: 10.4103/UA.UA_14_20. Epub 2021 Jan 19. PMID: 33897168; PMCID: PMC8052899.
o Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, Perry KT, Nadler RB. Update on the Diagnosis and Management of Renal Angiomyolipoma. J Urol. 2016 Apr;195(4 Pt 1):834-46. doi: 10.1016/j.juro.2015.07.126. Epub 2015 Nov 21. PMID: 26612197.
o Rofaiel G, Pan G, Campsen J, Kim R, Hamilton B. Successful Utilization of a Live Donor Kidney with Angiomyolipoma. Cureus. 2020 Feb 10;12(2):e6937. doi: 10.7759/cureus.6937. PMID: 32190489; PMCID: PMC7067347.
o UpToDate.
Exellent.
👉 I will accept the current donor only after confirmation of:
_ Being unilateral as the provided CT image illustrate the left kidney only.
_ MRI imaging of the abdomen is essential to detect low fat angiomyolipoma (AML) as epithelioid type has risk of malignant transformation (and not detected by CT).
⭐Being > 4 cm can preclude donation except if excision is feasable prior to transplantation to ensure it is forward AML.
_ Exclusion of the high risk for bleeding as large mass > 6 cm, intralesional aneurysm > 5 mm or annual increase in size > 2.5 mm, getting pregnant or use of hormonal contraceptives pills, presence of hypertension or use of anticoagulant therapy.
_Exclusion of high risk of malignant transformation as that associated with tuberous sclerosis (TS) or lung multiple angioleiomyomatosis.
_ as regard the size (bilateral lesions , unilateral lesion more than 6 cm preclude donation), size less than 4 cm can be used for donation with either excision pretramsplant or close follow up with US imaging after Insitu implantation
👉 AML is benign renal tumor that is composed of mixed muscle, fatty tissue and blood vessel component, more common in females, can be sporadic or associated with other conditions like TS. Can be rich in fat (good prognosis) or poor in fat (better delineation by MRI and has poor prognosis as it is of epithelioid nature).
👉 Risk associated are bleeding and malignant transformation into angioliposarcoma as mentioned above
👉 Other disease associated with AML are:
_ tuberous sclerosis (TS).
_Multiple angioleiomyomatosis.
_nurofibromatosis type 1.
The 1 st 2 conditions are associated with increased risk of malignant transformation.
Reference Up-to-date
Well done.
Will you accept this donor?
Donor HLA 3/6 mismatch no DSA with incidental finding of AML diameter 4.6cm.
Before we determine whether to accept or reject this donor we need to confirm that the donor doesn’t have tuberous sclerosis or pulmonary lymphangioleiomyomatosis or the AML is not the epithelial variant which are associated with malignancy.
If this is a sporadic AML then we can accept this donor.
What is angiomyolipoma?
They are begin renal neoplasm that contain fat, smooth muscles and vascular tissue.
They can occur either sporadically that are usually small, solitary and unilateral or in association of tuberous sclerosis that are usually bilateral, multiple and large.
What are the risks involved?
There is risk for malignancy especially for larger >7cm in diameter, with tumour necrosis and epitheliod carcinoma like pattern.
There is also risk of bleeding for large tumours, in women receiving hormonal therapy, presence of intralesional blood vessel or aneurysm dilatation and in uncontrolled hypertension.
Is this image adequate?
CT scan though a good modality for diagnosis its not able to differentiate fat poor and fat invisible AML.
MRI best for diagnosing fat poor AML.
Biopsy should be avoided due to risk of tumour rupture and haemorrhage.
Further imaging are thus required CT chest to rule out pulmonary LAM and imaging of contralateral kidney since in tuberous sclerosis they can have bilateral AML.
What other disease are associated with AML?
Tuberous sclerosis
Lymphangiomyomatosis
Neurofibromatosis
References
Medscape
Anton DG, Kovvuru K, Kanduri SR, Aeddula NR, Bathini T, Thongprayoon C, Kaewput W, Wijarnpreecha K, Watthanasuntorn K, Salim SA, Matemavi P, Vaitla P, Rivera FC, Cheungpasitporn W. Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urol Ann. 2021 Jan-Mar;13(1):67-72. doi: 10.4103/UA.UA_14_20. Epub 2021 Jan 19. PMID: 33897168; PMCID: PMC8052899.
Well done
1- Will you accept this donor?
Yes,I will. As unilateral angiomyolipoma is not contraindication for kidney donation ,but firther assessment is needed
2- What is angiomyolipoma?
Angiomyolipma is a benign tumer consists of smooth muscle cells, fat and thick vessels, It is more common infemales than males . It could be sporadic or associated with other conditions as tuberous sclerosis and lymphangiomyoliposis . It is not a precancerous lesion but commonly grow large and be complicated by retroperitoneal heamorrage that could be fatalin severe cases.
3- The risks involved are
– Bleeding : common to occur due to high vascularity of angiomyolipoma and in severe cases could be fatal .The risk of bleeding is increased in cases of large tumer (>6 cm) ,intratumer aneurysmaldilatation > 5 cm in diameter , uncontrolled hypertension , hormonaltherapy or pregnancy and treatment with anticoagulant drugs.
– Sarcomatous transformation : rare, could occur in cases associated with tuberous sclerosis
4- Further radiological evaluation is needed to assess whether this was a sporadic Angiomyolipoma or associated with tuberous sclerosis or lymphangiomyoliposis so we need MRI chest and pelvi abdomen .
5- Other diseases associated with angiomyolipoma are
– Tuberous sclerosis
– Lymphangiomyolipomatosis.
Ref
1- Anton, DesireeGarcia & Kovvuru, Karthik & Kanduri, Swetha Rani & Aeddula, NarothamaReddy & Bathini, Tarun & Thongprayoon, Charat & Kaewput, Wisit & Watthanasuntorn, Kanramon & Salim, SohailAbdul & Matemavi, Praise & Vaitla, Pradeep & Rivera, FrancoCabeza & Cheungpasitporn, Wisit. (2021). Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review. Urology Annals. 13. 67. 10.4103/UA.UA_14_20.
Did you consider it unilateral?
What is the role of CT, MRI.
In terms of immunological risks, this is a good donor with a three mismatch and no DSAa.
However, there is an incidental finding of an angiomyolipoma on the CT scan. Before we can accept or reject the donor, we need to get more history from the donor and more imaging studies need to be done. We need to assess the donor for history of tuberous sclerosis or any pulmonary symptoms which could suggest pulmonary lymphangioleiomyomatosis (LAM). He will need to get an MRI abdomen done as well as a CT scan chest. The risk here is whether this is malignant or it has a high probability of malignant transformation.
If this is a sporadic AML (ie not associated with TSC or pulmonary LAM), then it has a lower chance of being malignant or undergoing malignant transformation.
Then the brother can be used as a donor. The options for the transplant include:
A systematic review of 14 studies by Anton et al showed that use of AML kidneys with no TSC or pulmonary LAM had safe outcomes and good graft function.
Angiomyolipomas (AML) are triphasic tumors consisting of vascular tissue, myocytes and adipose tissue. Majority are benign and are more common in females than in males. Majority are asymptomatic and are discovered incidentally on imaging. Large tumors (more than 6 cm) can cause pain and can bleed leading to excruciating pain and sometimes hemorrhagic shock
Majority are sporadic (up to 80%) while 10-30% are associated with tuberous sclerosis complex, pulmonary LAM or both. There are two histological variants:
Classical variant – more common
Epithelioid variant – Associated with TSC or pulmonary LAM
They can also be classified as fat rich, fat poor or fat invisible.
The diagnosis of AML is made on imaging. US can pick up the masses but will need either a CT scan or an MRI to confirm the diagnosis
An MRI has a better sensitivity for detecting fat poor and fat invisible AMLs
Biopsies are rarely carried out due to the risk of tumor rupture. If the diagnosis is uncertain, the mass is excised and sent for histology
Risks with AML include:
1.Bleeding risks: Risk is higher with:
2.Malignant transformation
The other conditions associated with AML include:
Anton, et al.: Donors with renal angiomyolipoma
Well done very comprehensive review ,as you mentioned there are risk , predictive factors for malignant changes .
WHAT do you need to check here ,to takethe decision.
Thank you Prof
We shall need to make sure that it is not associated with tuberous sclerosis complex or pulmonary LAM
The size also matters as the larger the size (more than 6 cm), the higher risk of bleeding
this is not a good candidate for donation, and I will accept his kidney for donation.
As guideline says that;
Bilateral and lesion more than 4cm generally preclude living kidney donation but in case unilateral lesion more than 4cm can donate if ex vivo exclusion of the AML appears to be straightforward.
Incidental unilateral solitary AMLless than 4cm with specific CT scan feature and criteria doesn’t usually preclude donation.
Lesion less than 1cm can be consider doner of left in situ in the doner remaining kidney.
Lesion 1to 4 cm can be consider a doner and it depends upon its position, consideration of whether ex vivo excision of AMLis straightforward, or whether it can be left in situ in recipient and need serial ultrasound imaging
Angiomyolipoma is a benign cancer occurring in kidney and composed of varying amount of blood vessel resembling tissue, smooth muscles, and adipose tissue. Majority ae benign but malignant transformation are also reported
Risk of bleeding and transformation to malignant lesion
Above image is not adequate need Further imaging and if possible need tissue diagnosis.
Other disease association are tuberous sclerosis complex, sporadic lymphangioleiomyomatosis, chronic TB, RCC.
BTS/RA Living Donor Kidney Transplantation Guidelines 2018
Ferry JA, Malt RA, Young RH. Renal angiomyolipoma with sarcomatous transformation and pulmonary metastases. Am J Surg Pathol 1991; 15:1083.
Matute Martinez CF, Hamdan A, Sierra David JG, Kolli S. Sporadic, classic-type renal angiomyolipoma with renal vein and inferior vena cava extension: an incidental case. BMJ Case Rep 2021; 14.
Parekh V, Shen D. Invasive Renal Angiomyolipoma With Cytologic Atypia. Int J Surg Pathol 2017; 25:177.
Sato K, Ueda Y, Tachibana H, et al. Malignant epithelioid angiomyolipoma of the kidney in a patient with tuberous sclerosis: an autopsy case report with p53 gene mutation analysis. Pathol Res
mistake in typing i will not accept his kidney
Will you accept this donor?
No, I will not accept. AML >4 cm generally preclude living kidney donation (unless excision of the AML if possible appears to be straightforward)
AML more than 4 cm in diameter is more likely to have significant growth
BTS/RA Living Donor Kidney Transplantation Guidelines 2018:
Bilateral AML and AML >4 cm generally preclude living kidney donation although occasionally unilateral large (>4 cm) AML can be used if ex vivo excision of the AML appears to be straightforward.
An incidental, unilateral solitary AML <4 cm with typical characteristic CT criteria does not usually preclude donation.
A kidney with an AML <1 cm may be considered for donation or left in situ in the donor’s remaining kidney.
Kidneys containing a single AML between 1 and 4 cm can be considered for donation depending on its position, consideration of whether ex vivo excision of the AML is straightforward, or whether it can be left in situ in the recipient and followed with serial ultrasound imaging. (C1)
What is angiomyolipoma?
It is the most common benign renal tumour. AML Composed of mature adipose tissue, smooth muscle and thick walled blood vessels. Diagnosis can made by imaging with no need for biopsy if it is clear classic.
What are the risks involved?
Size > 4 cm: most likely to be symptomatic with significant growth
Calcification or necrosis: suspicion of RCC
Is this image adequate?
A well-defined mass in the renal cortex and containing fat with no necrosis or calcification is most likely to be AML. Must be reviewed by a uroradiologist
For donation, as it is > 4 cm, excision if possible is essential to make sure it is straightforward AML
What are the other diseases associated with angiomyolipoma?
1. Lymphangioleiomyomatosis
2. Neurofibromatosis type 1
3. Tuberous sclerosis complex
4. von Hippel-Lindau disease
References
1. BTS/RA Living Donor Kidney Transplantation Guidelines 2018.
Thank you for this excellent answer, do you think that this image is adequate?
It shows one kidney only
thank you prof.
Bilateral AML or unilateral > 4 cm, both of them are the same and preclude donation. No way of donation of bilateral AML, but there is a possibility of acceptance the unilteral one if it proved to be AML straightforward. so, the imaing is not adequate and should include the other kidney. Actually imaging should be for the chest, abdomen, and pelvis.
Will you accept this donor?
Yes; I will accept this donor. After discussion of the case with urologist and radiologist.
BTS guide lines 2018 stated:
– Contrast enhanced renal CT scan, ultrasound and / or MRI can usually distinguish between benign lesions such as angiomyolipoma (AML) or malignancy such as renal cell carcinoma (RCC). Review by a specialist uroradiologist is recommended.
– Bilateral AML and AML >4 cm generally preclude living kidney donation although occasionally unilateral large (>4 cm) AML can be used if ex vivo excision of the AML appears to be straightforward.
– An incidental, unilateral solitary AML < 4 cm usually doesn’t precludes donation.
– Single kidney AML <1 cm either donated or can be left in situ.
– Single AML between 1 and 4 cm can be considered for donation depending on its position, consideration of whether ex vivo excision of the AML is straightforward, or can be left in situ in the recipient and followed with serial ultrasound biannually.
However, Small asymptomatic lesions (<4 cm) remain stable, medium-sized lesions (4-8 cm) have variable behavior warranting serial imaging large lesions(>8 cm) are associated with significant morbidity and due to the potential complications should be electively treated.
What is angiomyolipoma?
Angiomyolipomas (AMLs) are neoplasms occurring in the kidney, composed of varying amounts of tissues, blood vessels, smooth muscle, and adipose tissue. The vast majority of AMLs are benign but malignant AMLs have been reported.
What are the risks involved?
1. Risk of bleeding, increases with large size AMLs, and in females recievning any hormonal therapy or at pregnancy.
2. Risk of malignant transformation, such as epithelioid AML.
Is this image adequate?
It is most probably adequate as it is contrast CT, which can identify the lesion. If the lesion is suspected to be fat-poor lesion, then MRI with gadolinium is more sensitive.
What are the other diseases associated with angiomyolipoma?
Tuberous sclerosis complex (TSC).
Sporadic lymphangioleiomyomatosis (LAM).
A combination (TSC-LAM).
References:
(1) Andrews PA, Burnapp L. British Transplantation Society / Renal Association UK Guidelines for Living Donor Kidney Transplantation 2018: Summary of Updated Guidance. Transplantation. 2018 Jul;102(7):e307. doi: 10.1097/TP.0000000000002253. PMID: 29688993; PMCID: PMC7228639.
(2) Gopalakrishnan N, Dhanapriya J, Sakthirajan R, Dineshkumar T, Balasubramaniyan T, Haris M. Angiomyolipoma of donor kidney: Successful transplantation and 5-year follow-up. Indian J Nephrol. 2016 Mar-Apr;26(2):131-3. doi: 10.4103/0971-4065.159552. PMID: 27051138; PMCID: PMC4795429.
(3) UpToDate- renal Angiomyolipomas (AMLs): epidemiology, pathogenesis, clinical amifestations, and diagnosis. Dec 2022.
Thank you for this excellent answer, do you think that this image is adequate?
It shows one kidney only
Thank you Prof. absolutely not adequate as i highlighted in the answer we should have the second kidney visualized in order to better decide on whether to accept this patient or not.
-Will you accept this donor?
-The answer can NO or YES as well:
-What is angiomyolipoma?
-What are the risks involved?
-Is this image adequate?
–What are the other diseases associated with angiomyolipoma?
Source: BTS-2018 guidelines, Cleveland clinic notes, oxford hand book of nephrology & hypertension 2 edition.
Thank you for this excellent answer, do you think that this image is adequate?
It shows one kidney only
Thank prof, Yes, it may not adequate because you may have lesion on the other kidney which is not seen in the image (i.e bilateral AML). This emphasize the important of a specialist uro-radiologist in the team
So, after telling the donor and the recipient about the risks and giving them advice, we should discuss with the surgeon whether or not we can remove the mass. I will accept the donor after ruling out syndromic causes and confirming the sporadic nature of AML and the lack of another available donor.
Angiomyolipomas, or AML, is non-encapsulated benign neoplasms made up of thickened blood vessels, smooth muscle, and fatty tissue that come from the mesenchymal parts of the kidney.
The prevalence of aberrant arteries and pseudoaneurysms makes hemorrhage the most common and serious consequence of highly vascular cancers like AML, particularly in larger tumors measuring more than 8 centimeters in diameter. It is well-known that AML may cause retroperitoneal hemorrhage, which is a complication that can occasionally result in mortality (“Wunderlich syndrome”). In very rare cases, patients with AML may have sarcomatous abnormalities that spread into the tissues nearby.
MRI with contrast to confirm the diagnosis (rich fat in AML).
A technetium-99m renogram is used to determine the total glomerular filtration rate. multislice CT to check the other kidney. CT chest and MRI brain. The diagnosis of sporadic AML should be confirmed, and syndromic causes should be ruled out.
-TSC
– lymphangioleiomyomatosis (LAM)
Reference:
-Dhanapriya J, Gopalakrishnan N, Sakthirajan R, Dineshkumar T, Balasubramaniyan T, Md. Haris. Angiomyolipoma of donor’s kidney: successful transplantation and 5-year follow-up. Indian Journal of Nephrology. 2016;26(2):131.
-British Guidelines for living donor kidney transplantation2018
Thank you for this excellent answer, do you think that this image is adequate?
It shows one kidney only
MRI with contrast to confirm the diagnosis (rich fat in AML).
A technetium-99m renogram is used to determine the total glomerular filtration rate. multislice CT to check the other kidney.
I will accept the donor with plan to harvest the kidney with Angiomyolipoma AML.
AML is a benign tumor that has propensity to enlarge and bear a significant risk of inflicting retroperitoneal hemorrhage. The transplantation is usually including removal of the tumor in a back table and then transplant a kidney without tumor.
AML is a benign tumor with triphasic or typical monophasic component.
Generally is containing dilated blood vessels, myocytes and adipose cells. Nevertheless, some tumors are emerged with predominantly one component and minute amount of other types. radiological detection of Fat inside the tumor is the sine gua non of AML.
Fundamentally, its benign tumor that inherently involve two major consequences’,
1) relentless enlargement of the tumor.
2) Risk of retroperitoneal hemorrhage.
Classically vasculature of AML has to be identified and outlined pre-operatively in order to resect the tumor properly.
In 80 % its isolated tumors.
in 20 % its part of Tuberusclerosis complex TSC and pulmonary lymphangioleiomyomatosis.
references:
1)Successful Utilization of a Live Donor Kidney with Angiomyolipoma.George Rofaiel et al.Cureus. 2020 Feb; 12(2): e6937.2) Renal Angiomyolipoma: The Good, the Bad, and the Ugly.Nicolas Vos and Raymond Oyen.J Belg Soc Radiol. 2018; 102(1): 41.
Thank you for this excellent answer, do you think that this image is adequate?
It shows one kidney only
· Will you accept this donor?
YES.
· Bilateral AML and AML >4 cm generally preclude living kidney donation although occasionally unilateral large (>4 cm) AML can be used if ex vivo excision of the AML appears to be straightforward.
· An incidental, unilateral solitary AML with typical characteristic CT criteria does not usually preclude donation.
· A kidney with an AML 1 and 4 cm can be considered for donation depending on its position consideration of whether ex vivo excision of the AML is straightforward or whether it can be left in situ in the recipient and followed with serial ultrasound imaging.(1)
What’s angiomyolipomas?
Renal angiomyolipomas (AMLs):
Are considered the most prevalent benign renal tumor.
Although considered benign, the tumors may extend into the surrounding perirenal fat or renal sinus and nearby organs and lymphatics.(2)
Pathology:
Highly vascular, primarily composed of smooth muscle and adipose tissues, and are distinguished by perivascular epithelioid differentiation.
Clinical feature:
Although most of these tumors are found incidentally on radiological imaging, symptomatic presentations also exist. .Typical symptoms would include flank pain, gross hematuria, and retroperitoneal hemorrhage, which can be severe.
Etiology:
Most sporadic.
· What are the other diseases associated with angiomyolipomas?
Hereditary conditions (20%) such as tuberous sclerosis and pulmonary
lymphangioleiomyomatosis.
Currently, the European Association of Urology and the Canadian Urological Association recommend that intervention is required in well-selected cases and should consider the following criteria:
· What are the risks involved?
· Renal angiomyolipomas that contain aneurysms >5 mm or the size of the tumor is larger than 6 cm pose a significant risk of spontaneous rupture and bleeding, which is considered a life-threatening condition.
· Some types of angiomyolipomas have a greater tendency for this transformation to malignancy.
· Is this image adequate?
NO.
MRI yearly to monitor the condition because of its higher sensitivity in detecting adipose tissue
References:
1-BTS GUIDLINE 2018.
2- Younus M. Shamam; Stephen W. Lesl.Renal Angiomyolipoma. StatPearls Publishing;
2022 Jan-.
Thank you for this excellent answer, do you think that this image is adequate?
It shows one kidney only
Thank Prof.
No idont think its adequate ,cause both kidney should be seen as bilateral AML
will exclude the donors.
Will you accept this donor?
What is angiomyolipoma?
What are the risks involved?
A- Hemorrhage is the most common and most feared complication; the presence of one of the following is associated with high risk of bleeding
B- Wunderlich syndrome which is defined as non-traumatic renal hemorrhage into perirenal and subcapsular spaces may occur as a result of AML, it is a life-threatening condition (4).
C- Malignant transformation is rare but it can occur in epithelioid AMLs
D- Extensive bilateral AMLS such as that occurring in TSC may cause destruction of renal tissues and ESRD
Is this image adequate? Diagnosis
What are the other diseases associated with angiomyolipoma?
Angiomyolipoma occur mostly sporadic (>80% of cases) or in association with certain diseases
REFERANCES
Thank you for this excellent answer, do you think that this image is adequate?
It shows one kidney only
· Will you accept this donor?
Yes after MDT evaluation and if accepted will underwent surgical excision before transplantation
What is angiomyolipoma
Renal angiomyolipoma (AML) is the most frequent mesenchymal tumor of the kidney.[1] Although first referenced in 1900its histopathology was originally described by Fischer in 1911. ,[2] Renal AML is a heterogeneous, triphasic tumor with varying elements of smooth muscle, adipose tissue, and vascular elements.[3]
What are the risks involved?
With tumors >4 cm
potential vascular aneurysm, that my rupture, hemorrhage (Wunderlich syndrome) putting the patient in emergency situation
compression of renal parenchyma further contributes to chronic kidney disease with worsening renal failure,
urinary concentration defects,
essential hypertension.
Malignant transformation
· Is this image adequate?
This image is CT abdomen that can not differentiate between AML poor fat and invisible fat AML .
Better soft tissue mass evaluation by MRI with contrast
· What are the other diseases associated with angiomyolipoma?
Renal AML can occur sporadically with an incidence ranging from 55% to 80%or in association with the tuberous sclerosis complex (TSC) in about 20%–30% and very rarely as sporadic lymphangioleiomyomatosis (LAM).[4]
Ref
1. Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, et al Update on the diagnosis and management of renal angiomyolipoma J Urol. 2016;195:834–46
2. Nelson CP, Sanda MG. Contemporary diagnosis and management of renal angiomyolipoma J Urol. 2002;168:1315–25
3- Sampson JR. The kidney in tuberous sclerosis: Manifestations and molecular genetic mechanisms Nephrol Dial Transplant. 1996;11(Suppl 6):34–7
4- Meraj R, Wikenheiser-Brokamp KA, Young LR, McCormack FX. Lymphangioleiomyomatosis: New concepts in pathogenesis, diagnosis, and treatment Semin Respir Crit Care Med. 2012;33:486–97
Thank you for this excellent answer, do you think that this image is adequate?
It shows one kidney only
WILL U ACCEPT THIS DONOR?
Apparantly NO
WHAT IS ANGIOMYOLIPOMA?
Angiomyolipomas (AMLs) are neoplasms occurring in the kidney that are composed of varying amounts of tissues resembling blood vessels, smooth muscle, and adipose tissue. The vast majority of AMLs are benign but malignant AMLs have been reported.
WHAT ARE THE RISKS INVOLVED?
Hematuria
Loin pain
Small risk of malignant transformation
IS THIS IMAGE ADEQUATE.?
NO
We preferably use MRI to establish the diagnosis of renal AML. Although not necessary to diagnose AML, administration of gadolinium contrast is used to determine the vascularity of the lesion and detect intratumoral aneurysms. When an MRI is not available or is inappropriate (eg, if the patient has contraindications to MRI or is unable to remain still for the duration of an MRI), then we perform unenhanced and contrast-enhanced CT. We perform an MRI (or CT) even among patients who had the diagnosis of AML suggested by ultrasound.(up to date)
WHAT ARE THE OTHER DISEASES ASSOCIATED WITH ANGIOMYOLIPOMA?
Some patients with AML may need additional evaluation for for tuberous sclerosis complex and lymphangioleiomyomatosis in some patients
We evaluate all patients with newly diagnosed AML for possible TSC.
The extent of the evaluation depends on the index of suspicion for TSC:
Patients with multiple (three or more), bilateral, or larger (ie, ≥4 cm) AMLs are more likely to have TSC. Among such patients, we perform a complete evaluation for TSC (which includes both a clinical evaluation and genetic testing). The diagnostic criteria for TSC are based upon specific clinical features
Thank you for this excellent answer, do you think that this image is adequate?
It shows one kidney only
Will you accept this donor?
What is angiomyolipoma?
What are the risks involved?
Is this image adequate?
What are the other diseases associated with angiomyolipoma?
Short and sweet, well done. We should have a full CT to see the other kidney also.
Will you accept this donor?
///////////////////////////////
What is angiomyolipoma?
Renal angiomyolipoma (AML):
Occurrence:
Clinical features:
///////////////////////////////
What are the risks involved?
///////////////////////////////
Is this image adequate?
No
///////////////////////////////
What are the other diseases associated with angiomyolipoma?
References
Thank you for this excellent answer, do you think that this image is adequate?
It shows one kidney only
History
====================================================================
1- Will you accept this donor?
Yes I will accept this donor
====================================================================
2- What is angiomyolipoma?
====================================================================
3- What are the risks involved?
===================================================================
4- Is this image adequate?
Ultrasound
CT
MRI
Digital subtraction angiography (DSA)
A- arterial phase:
a sharply marginated hypervascular mass with a dense early arterial network, and tortuous vessels giving the “sunburst” appearance.
B- venous phase:
whorled “onion peel” appearance of peripheral vessels
===================================================================
What are the other diseases associated with angiomyolipoma?
====================================================================
Conclusion
===================================================================
Refernce
1-M.S. Steiner, S.M. Goldman, E.K. Fishman, F.F. MarshallThe natural history of renal angiomyolipoma Journal of Urology, 150 (6) (1993), pp. 1782-1786
2- P.A. Hellström, A. Mehik, M.T. Talja, T.M. Siniluoto, J.M. Perälä, S.S. Leinonen
Spontaneous subcapsular or perirenal haemorrhage caused by renal tumours. A urological emergency Scandinavian Journal of Urology and Nephrology, 33 (1) (1999),pp. 17-23
3- Nelson CP, Sanda MG. Contemporary diagnosis and management of renal angiomyolipoma. J Urol. 2002;168(4 Pt 1):1315.
4-Sampson JR. The kidney in tuberous sclerosis: Manifestations
and molecular genetic mechanisms. Nephrol Dial Transplant
1996;11 Suppl 6:34-7.
Thank you for this excellent answer, do you think that this image is adequate?
It shows one kidney only
Thanks alot for you Prorf.Halawa
BTS/RA Living Donor Kidney Transplantation Guidelines 2018 159
1. Yes, I will accept donor.
2. Angiomyolipomas (AMLs) are neoplasms occurring in the kidney, comprising of varying amounts of tissues which resemble blood vessels, smooth muscle, and adipose tissue. Most AMLs are benign.
3. Common presentation: Flank pain, retroperitoneal haemorrhage, or recurrent episodes of gross haematuria.
Patients may present with impaired renal function due to slow, chronic impingement of the AMLs on normal tissue.
4. Diagnostic evaluation: Ultrasound, CT scan, MRI: presence of fat in the AML lesion.
5. For minimal fat or fat-poor lesions, image-guided percutaneous needle biopsy should be performed.
6. Associations: Tuberous sclerosis complex (TSC)
Lymphangioleiomyomatosis (LAM)
Thank you for this excellent answer, do you think that this image is adequate?
It shows one kidney only
1- yes will accept this donor
2- angiomyolipoma AML of the kidney is a mesenchymal benign tumor and it is common , it is harmless, with no evidence of malignant transformation, can cause retroperitoneal hemorrhage, so, should be excised from the donor kidney before transplantation.
3-risks involved is retroperitoneal bleeding and tumor related complications
4- unenhanced CT and a T2-hypointense appearance at MRI both correspond to the smooth muscle component, are important diagnostic clues to the types of angiomyolipomas that contain few or no fat cells. Because malignancies can also demonstrate these imaging characteristics, percutaneous biopsy is recommended for small (less than or equal to 3 cm) hyperattenuating, T2-hypointense, enhancing renal masses so that unnecessary surgery can be avoided.
When hyperattenuating, T2-hypointense, enhancing renal masses are larger, or there is evidence of massive hemorrhage, proceeding directly to surgery may be appropriate, both to prevent further bleeding and because both renal cancer and epithelioid AML are more likely.
5-tuberous sclerosis complex (TSC)
references:
1- N. Gopalakrishnan, J. Dhanapriya, R. Sakthirajan, T. Dineshkumar, T. Balasubramaniyan, Md. Haris. Angiomyolipoma of donor kidney: Successful transplantation and 5-year follow-up. Indian J Nephrol. 2016 Mar-Apr; 26(2): 131–133.
2-George Rofaiel,Gilbert Pan,Jeffrey Campsen, Robin Kim,Blake Hamilton. Successful Utilization of a Live Donor Kidney with Angiomyolipoma. Cureus. 2020 Feb; 12(2): e6937.
3-H.AbboudiaP.ChandakaN.KessarisaJ.Froneka. A successful live donor kidney transplantation after large angiomyolipoma excision. International Journal of Surgery Case Reports.Volume 3, Issue 12, 2012, Pages 594-596
4- Masahiro Jinzaki,Stuart G. Silverman, Hirotaka Akita, Yoji Nagashima, Shuji Mikami, Mototsugu Oya.Renal angiomyolipoma: a radiological classification and update on recent developments in diagnosis and management.Abdom Imaging. 2014; 39(3): 588–604.
5- Canbin Lin, Lu Jin, Yu Yang, Yu Ding, Xionghui Wu, Liangchao Ni, Shangqi Yang,Yongqing Lai. Tuberous sclerosis-associated renal angiomyolipoma: A report of two cases and review of the literature. Mol Clin Oncol. 2017 Oct; 7(4): 706–708.
Thank you for this excellent answer, do you think that this image is adequate?
It shows one kidney only
should show both kidneys as bilateral multiple AML is associated with tuberous sclerosis
so both kidneys should be seen in the image
Sushma Shankar, Kiran Shankar Hulikanthimatt, Shreeharsha Mallappa Awatti, Suresh Turuvekere Narayanrao. Bilateral Multifocal Renal Angiomyolipoma Associated with Wunderlich’s Syndrome in A Tuberous Sclerosis Patient. J Clin Diagn Res. 2015 Aug; 9(8): ED01–ED03.
H B Tongaonkar , M B Sampat, A V Dalal, N P Dandekar, J N Kulkarni, M R Kamat. Bilateral renal angiomyolipoma. J Surg Oncol.1994 Sep;57(1):65-70.
Surely, one will be interested to see what is going on in the other side, rather than assuming that it is normal/