Spectrum of Presentations and Management Strategies in Renal Angiomyolipoma
Main Article Content
Keywords
angiomyolipoma, epithelioid renal angiomyolipoma, mTOR inhibitors, PEComa
Abstract
Renal angiomyolipoma (rAML) occurs rarely sporadically but is commonly encountered in patients with tuberous sclerosis complex and lymphangioleiomyomatosis. rAML is a rare entity, not seen regularly in daily practice; however, is commonly encountered and diagnosed by clinicians who approach and treat kidney masses. Basic knowledge of this entity is necessary to recognize that despite being benign, these tumors can rarely cause deadly complications such as hemorrhage or severe renal dysfunction or may have malignant components associated with them.
References
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4. Park SG, Park BK. New radiologic classification of renal angiomyolipoma: Frequently asked questions. Clin Imaging. 2019;55:156–60. 10.1016/j.clinimag.2019.01.025
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10. Rule AD, Sasiwimonphan K, Lieske JC, Keddis MT, Torres VE, Vrtiska TJ. Characteristics of renal cystic and solid lesions based on contrast-enhanced computed tomography of potential kidney donors. Am J Kidney Dis. 2012;59(5):611–18. 10.1053/j.ajkd.2011.12.022
11. Koo KC, Kim WT, Ham WS, Lee JS, Ju HJ, Choi YD. Trends of presentation and clinical outcome of treated renal angiomyolipoma. Yonsei Med J. 2010;51(5):728–34. 10.3349/ymj.2010.51.5.728
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17. Maclean DFW, Sultana R, Radwan R, McKnight L, Khastgir J. Is the follow-up of small renal angiomyolipomas a necessary precaution? Clin Radiol. 2014;69(8):822–6. 10.1016/j.crad.2014.03.016
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19. Armah HB, Yin M, Rao UN, Parwani AV. Angiomyolipoma with epithelial cysts (AMLEC): A rare but distinct variant of angiomyolipoma. Diagn Pathol. 2007;2:11. 10.1186/1746-1596-2-11
20. Wei J, Li Y, Wen Y, Li L, Zhang R. Renal angiomyolipoma with epithelial cysts: A rare entity and review of literature. Int J Clin Exp Pathol. 2015;8(9):11760–5. eCollection 2015.
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22. Woo S, Cho JY. Imaging findings of common benign renal tumors in the era of small renal masses: Differential diagnosis from small renal cell carcinoma: Current status and future perspectives. Korean J Radiol. 2015;16(1):99–113. 10.3348/kjr.2015.16.1.99
23. Park BK. Renal angiomyolipoma: Radiologic classification and imaging features according to the amount of fat. Am J Roentgenol. 2017;209(4):826–35. 10.2214/AJR.17.17973
24. Liu J, Bao J, Zhang W, Li Q, Hou J, Wei X, et al. The potential of visceral adipose tissue in distinguishing clear cell renal cell carcinoma from renal angiomyolipoma with minimal fat. Cancer Manag Res. 2021;13:8907–14. 10.2147/CMAR.S336920
25. Nese N, Martignoni G, Fletcher CD, Gupta R, Pan C-C, Kim H, et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: Detailed assessment of morphology and risk stratification. Am J Surg Pathol. 2011;35(2):161–76. 10.1097/PAS.0b013e318206f2a9
26. Lee W, Choi SY, Lee C, Yoo S, You D, Jeong IG, et al. Does epithelioid angiomyolipoma have poorer prognosis, compared with classic angiomyolipoma? Investig Clin Urol. 2018;59(6):357–62. 10.4111/icu.2018.59.6.357
27. Brimo F, Robinson B, Guo C, Zhou M, Latour M, Epstein JI. Renal epithelioid angiomyolipoma with atypia: A series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. Am J Surg Pathol. 2010;34(5):715–22. 10.1097/PAS.0b013e3181d90370
28. Pacella G, Faiella E, Altomare C, Andresciani F, Castiello G, Bernetti C, et al. Different Treatments of Symptomatic Angiomyolipomas of the Kidney: Two Case Reports. J Kidney Cancer VHL. 2021 Oct 19;8(4):32–7. Different treatments of symptomatic angiomyolipomas of the kidney: Two case reports. J Kidney Cancer VHL. 2021;8(4):32–7. 10.15586/jkcvhl.v8i4.181
29. Chapman D, Tyson M, Buckley B. Single-institution, retrospective review of elective and emergency embolization of renal angiomyolipoma. Can Urol Assoc J. 2021;15(11):E598–E602. 10.5489/cuaj.7143
30. Davis C, Boyett T, Caridi J. Renal artery embolization: Application and success in patients with renal cell carcinoma and angiomyolipoma. Semin Interv Radiol. 2007;24(1):111–16. 10.1055/s-2007-971185
31. Sobiborowicz A, S´witaj T, Teterycz P, Spałek MJ, Szumera-Ciec´kiewicz A, Wa˛grodzki M, et al. Feasibility and long-term efficacy of PEComa treatment–20 years of experience. J Clin Med. 2021;10(10):2200. 10.3390/jcm10102200
32. Osei DA, Alvandi F, Brooks JS, Ogilvie CM. PEComa of the upper extremity: A unique case and description of an initial response to neoadjuvant chemotherapy. Sarcoma. 2007;2007:53056. 10.1155/2007/53056
33. Kenerson H, Folpe AL, Takayama TK, Yeung RS. Activation of the mTOR pathway in sporadic angiomyolipomas and other perivascular epithelioid cell neoplasms. Hum Pathol. 2007;38(9):1361–71. 10.1016/j.humpath.2007.01.028
34. Sanfilippo R, Jones RL, Blay JY, Cesne AL, Provenzano S, Antoniou G, et al. Role of chemotherapy, VEGFR inhibitors, and mTOR inhibitors in advanced perivascular epithelioid cell tumors (PEComas). Clin Cancer Res. 2019;25(17):5295–300. 10.1158/1078-0432.CCR-19-0288
35. S´witaj T, Sobiborowicz A, Teterycz P, Klimczak A, Makuła D, Wa˛grodzki M, et al. Efficacy of sirolimus treatment in PEComa–10 years of practice perspective. J Clin Med. 2021;10(16):3705. 10.3390/jcm10163705
36. Wagner AJ, Ravi V, Riedel RF, Ganjoo K, Van Tine BA, Chugh R, et al. nab-Sirolimus for patients with malignant perivascular epithelioid cell tumors. J Clin Oncol. 2021;39(33):3660–70. 10.1200/JCO.21.01728
37. Fetsch PA, Fetsch JF, Marincola FM, Travis W, Batts KP, Abati A. Comparison of melanoma antigen recognized by T cells (MART-1) to HMB-45: Additional evidence to support a common lineage for angiomyolipoma, lymphangiomyomatosis, and clear cell sugar tumor. Mod Pathol Off J U S Can Acad Pathol Inc. 1998;11(8):699–703.
38. Lattanzi M, Deng FM, Chiriboga LA, Femia AN, Meehan SA, Iyer G, et al. Durable response to anti-PD-1 immunotherapy in epithelioid angiomyolipoma: A report on the successful treatment of a rare malignancy. J Immunother Cancer. 2018;6(1):97. 10.1186/s40425-018-0415-x
39. McBride A, Garcia AJ, Sanders LJ, Yiu K, Cranmer LD, Kuo PH, et al. Sustained response to pembrolizumab in recurrent perivascular epithelioid cell tumor with elevated expression of programmed death ligand: A case report. J Med Case Reports. 2021;15(1):400. 10.1186/s13256-021-02997-x
40. Bissler JJ, Kingswood JC, Radzikowska E, Zonnenberg BA, Frost M, Belousova E, et al. Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): A multicentre, randomised, double-blind, placebo-controlled trial. Lancet. 2013;381(9869):817–24. 10.1016/S0140-6736(12)61767-X
41. Everolimus: New indication in renal angiomyolipoma associated with tuberous sclerosis complex – The ASCO post. [cited 2022 Jan 3]. Available from: https://ascopost.com/issues/july-1-2012/everolimus-new-indication-in-renal-angiomyolipoma-associated-with-tuberous-sclerosis-complex/
42. Luo C, Ye WR, Zu XB, Chen M-F, Qi L, Li Y-L, et al. Low-dose everolimus maintenance therapy for renal angiomyolipoma associated with tuberous sclerosis complex. Front Med. 2021;8:744050. 10.3389/fmed.2021.744050
43. Gould Rothberg BE, Grooms MC, Dharnidharka VR. Rapid growth of a kidney angiomyolipoma after initiation of oral contraceptive therapy. Obstet Gynecol. 2006;108(3 Pt 2):734–6. 10.1097/01.AOG.0000188062.13749.ef
44. Zapardiel I, Delafuente-Valero J, Bajo-Arenas JM. Renal angiomyolipoma during pregnancy: Review of the literature. Gynecol Obstet Invest. 2011;72(4):217–19. 10.1159/000329328
45. Le P, Garg A, Brandao G, Abu-Sanad A, Panasci L. Hormonal manipulation with letrozole in the treatment of metastatic malignant pecoma. Curr Oncol. 2014;21(3):e518–20. 10.3747/co.21.1849
46. Chan K, Chedgy E, Bent C, Turner K. Surveillance imaging for sporadic renal angiomyolipoma less than 40 mm: Lessons learnt and recommendations from the experience of a large district general hospital. Ann R Coll Surg Engl. 2018;100(6):480–4. 10.1308/rcsann.2018.0040
2. Krajewski KM, Pedrosa I. Imaging advances in the management of kidney cancer. J Clin Oncol. 2018;36(36):3582–90. 10.1200/JCO.2018.79.1236
3. Vos N, Oyen R. Renal angiomyolipoma: The good, the bad, and the ugly. J Belg Soc Radiol. 2018;102(1):41. 10.5334/jbsr.1536
4. Park SG, Park BK. New radiologic classification of renal angiomyolipoma: Frequently asked questions. Clin Imaging. 2019;55:156–60. 10.1016/j.clinimag.2019.01.025
5. Thway K, Fisher C. PEComa: Morphology and genetics of a complex tumor family. Ann Diagn Pathol. 2015;19(5):359–68. 10.1016/J.ANNDIAGPATH.2015.06.003
6. Lam HC, Siroky BJ, Henske EP. Renal disease in tuberous sclerosis complex: Pathogenesis and therapy. Nat Rev Nephrol. 2018;14(11):704–16. 10.1038/s41581-018-0059-6
7. Akumalla S, Madison R, Lin DI, Schrock AB, Yakirevich E, Rosenzweig M, et al. Characterization of clinical cases of malignant PEComa via comprehensive genomic profiling of DNA and RNA. Oncology. 2020;98(12):905–12. 10.1159/000510241
8. Nelson CP, Sanda MG. Contemporary diagnosis and management of renal angiomyolipoma. J Urol. 2002;168(4 Pt 1):1315–25. 10.1097/01.ju.0000028200.86216.b2
9. Fittschen A, Wendlik I, Oeztuerk S, Kratzer W, Akinli AS, Haenle MM, et al. Prevalence of sporadic renal angiomyolipoma: A retrospective analysis of 61,389 in-and out-patients. Abdom Imaging. 2014;39(5):1009–13. 10.1007/s00261-014-0129-6
10. Rule AD, Sasiwimonphan K, Lieske JC, Keddis MT, Torres VE, Vrtiska TJ. Characteristics of renal cystic and solid lesions based on contrast-enhanced computed tomography of potential kidney donors. Am J Kidney Dis. 2012;59(5):611–18. 10.1053/j.ajkd.2011.12.022
11. Koo KC, Kim WT, Ham WS, Lee JS, Ju HJ, Choi YD. Trends of presentation and clinical outcome of treated renal angiomyolipoma. Yonsei Med J. 2010;51(5):728–34. 10.3349/ymj.2010.51.5.728
12. Kruseová J, Gottfriedová B, Zichová A, Švojgr K, Hošek P, Lukš A, et al. Is there a higher incidence of sporadic renal angiomyolipoma in childhood cancer survivors? Clin Epidemiol. 2021;13:707–16. 10.2147/CLEP.S317903
13. Guo Y, Kapoor A, Cheon P, So AI, Lattouf JB, Jamal M. Canadian Urological Association best practice report: Diagnosis and management of sporadic angiomyolipomas. Can Urol Assoc J. 2020;14(11):E527–36. 10.5489/cuaj.6942
14. Bhatt JR, Richard PO, Kim NS, Finelli A, Manickavachagam K, Legere L, et al. Natural history of renal angiomyolipoma (AML): Most patients with large AMLs >4cm can be offered active surveillance as an initial management strategy. Eur Urol. 2016;70(1):85–90. 10.1016/j.eururo.2016.01.048
15. Arslan B, Gürkan O, Çetin B, Arslan ÖA, Göv T, Yazıcı G, et al. Evaluation of ABO blood groups and blood-based biomarkers as a predictor of growth kinetics of renal angiomyolipoma. Int Urol Nephrol. 2018;50(12):2131–7. 10.1007/s11255-018-2012-9
16. Oesterling JE, Fishman EK, Goldman SM, Marshall FF. The management of renal angiomyolipoma. J Urol [Internet]. 1986 [cited 2022 Jan 9]. Available from: https://www.auajournals.org/doi/abs/10.1016/S0022-5347%2817%2946013-7
17. Maclean DFW, Sultana R, Radwan R, McKnight L, Khastgir J. Is the follow-up of small renal angiomyolipomas a necessary precaution? Clin Radiol. 2014;69(8):822–6. 10.1016/j.crad.2014.03.016
18. Çalıs¸kan S, Gümrükçü G, Özsoy E, Topaktas R, Öztürk MIfi. Renal angiomyolipoma. Rev Assoc Médica Bras. 2019;65:977–81. 10.1590/1806-9282.65.7.977
19. Armah HB, Yin M, Rao UN, Parwani AV. Angiomyolipoma with epithelial cysts (AMLEC): A rare but distinct variant of angiomyolipoma. Diagn Pathol. 2007;2:11. 10.1186/1746-1596-2-11
20. Wei J, Li Y, Wen Y, Li L, Zhang R. Renal angiomyolipoma with epithelial cysts: A rare entity and review of literature. Int J Clin Exp Pathol. 2015;8(9):11760–5. eCollection 2015.
21. 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(1):78–82. 10.1148/radiol.2251011477
22. Woo S, Cho JY. Imaging findings of common benign renal tumors in the era of small renal masses: Differential diagnosis from small renal cell carcinoma: Current status and future perspectives. Korean J Radiol. 2015;16(1):99–113. 10.3348/kjr.2015.16.1.99
23. Park BK. Renal angiomyolipoma: Radiologic classification and imaging features according to the amount of fat. Am J Roentgenol. 2017;209(4):826–35. 10.2214/AJR.17.17973
24. Liu J, Bao J, Zhang W, Li Q, Hou J, Wei X, et al. The potential of visceral adipose tissue in distinguishing clear cell renal cell carcinoma from renal angiomyolipoma with minimal fat. Cancer Manag Res. 2021;13:8907–14. 10.2147/CMAR.S336920
25. Nese N, Martignoni G, Fletcher CD, Gupta R, Pan C-C, Kim H, et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: Detailed assessment of morphology and risk stratification. Am J Surg Pathol. 2011;35(2):161–76. 10.1097/PAS.0b013e318206f2a9
26. Lee W, Choi SY, Lee C, Yoo S, You D, Jeong IG, et al. Does epithelioid angiomyolipoma have poorer prognosis, compared with classic angiomyolipoma? Investig Clin Urol. 2018;59(6):357–62. 10.4111/icu.2018.59.6.357
27. Brimo F, Robinson B, Guo C, Zhou M, Latour M, Epstein JI. Renal epithelioid angiomyolipoma with atypia: A series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. Am J Surg Pathol. 2010;34(5):715–22. 10.1097/PAS.0b013e3181d90370
28. Pacella G, Faiella E, Altomare C, Andresciani F, Castiello G, Bernetti C, et al. Different Treatments of Symptomatic Angiomyolipomas of the Kidney: Two Case Reports. J Kidney Cancer VHL. 2021 Oct 19;8(4):32–7. Different treatments of symptomatic angiomyolipomas of the kidney: Two case reports. J Kidney Cancer VHL. 2021;8(4):32–7. 10.15586/jkcvhl.v8i4.181
29. Chapman D, Tyson M, Buckley B. Single-institution, retrospective review of elective and emergency embolization of renal angiomyolipoma. Can Urol Assoc J. 2021;15(11):E598–E602. 10.5489/cuaj.7143
30. Davis C, Boyett T, Caridi J. Renal artery embolization: Application and success in patients with renal cell carcinoma and angiomyolipoma. Semin Interv Radiol. 2007;24(1):111–16. 10.1055/s-2007-971185
31. Sobiborowicz A, S´witaj T, Teterycz P, Spałek MJ, Szumera-Ciec´kiewicz A, Wa˛grodzki M, et al. Feasibility and long-term efficacy of PEComa treatment–20 years of experience. J Clin Med. 2021;10(10):2200. 10.3390/jcm10102200
32. Osei DA, Alvandi F, Brooks JS, Ogilvie CM. PEComa of the upper extremity: A unique case and description of an initial response to neoadjuvant chemotherapy. Sarcoma. 2007;2007:53056. 10.1155/2007/53056
33. Kenerson H, Folpe AL, Takayama TK, Yeung RS. Activation of the mTOR pathway in sporadic angiomyolipomas and other perivascular epithelioid cell neoplasms. Hum Pathol. 2007;38(9):1361–71. 10.1016/j.humpath.2007.01.028
34. Sanfilippo R, Jones RL, Blay JY, Cesne AL, Provenzano S, Antoniou G, et al. Role of chemotherapy, VEGFR inhibitors, and mTOR inhibitors in advanced perivascular epithelioid cell tumors (PEComas). Clin Cancer Res. 2019;25(17):5295–300. 10.1158/1078-0432.CCR-19-0288
35. S´witaj T, Sobiborowicz A, Teterycz P, Klimczak A, Makuła D, Wa˛grodzki M, et al. Efficacy of sirolimus treatment in PEComa–10 years of practice perspective. J Clin Med. 2021;10(16):3705. 10.3390/jcm10163705
36. Wagner AJ, Ravi V, Riedel RF, Ganjoo K, Van Tine BA, Chugh R, et al. nab-Sirolimus for patients with malignant perivascular epithelioid cell tumors. J Clin Oncol. 2021;39(33):3660–70. 10.1200/JCO.21.01728
37. Fetsch PA, Fetsch JF, Marincola FM, Travis W, Batts KP, Abati A. Comparison of melanoma antigen recognized by T cells (MART-1) to HMB-45: Additional evidence to support a common lineage for angiomyolipoma, lymphangiomyomatosis, and clear cell sugar tumor. Mod Pathol Off J U S Can Acad Pathol Inc. 1998;11(8):699–703.
38. Lattanzi M, Deng FM, Chiriboga LA, Femia AN, Meehan SA, Iyer G, et al. Durable response to anti-PD-1 immunotherapy in epithelioid angiomyolipoma: A report on the successful treatment of a rare malignancy. J Immunother Cancer. 2018;6(1):97. 10.1186/s40425-018-0415-x
39. McBride A, Garcia AJ, Sanders LJ, Yiu K, Cranmer LD, Kuo PH, et al. Sustained response to pembrolizumab in recurrent perivascular epithelioid cell tumor with elevated expression of programmed death ligand: A case report. J Med Case Reports. 2021;15(1):400. 10.1186/s13256-021-02997-x
40. Bissler JJ, Kingswood JC, Radzikowska E, Zonnenberg BA, Frost M, Belousova E, et al. Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): A multicentre, randomised, double-blind, placebo-controlled trial. Lancet. 2013;381(9869):817–24. 10.1016/S0140-6736(12)61767-X
41. Everolimus: New indication in renal angiomyolipoma associated with tuberous sclerosis complex – The ASCO post. [cited 2022 Jan 3]. Available from: https://ascopost.com/issues/july-1-2012/everolimus-new-indication-in-renal-angiomyolipoma-associated-with-tuberous-sclerosis-complex/
42. Luo C, Ye WR, Zu XB, Chen M-F, Qi L, Li Y-L, et al. Low-dose everolimus maintenance therapy for renal angiomyolipoma associated with tuberous sclerosis complex. Front Med. 2021;8:744050. 10.3389/fmed.2021.744050
43. Gould Rothberg BE, Grooms MC, Dharnidharka VR. Rapid growth of a kidney angiomyolipoma after initiation of oral contraceptive therapy. Obstet Gynecol. 2006;108(3 Pt 2):734–6. 10.1097/01.AOG.0000188062.13749.ef
44. Zapardiel I, Delafuente-Valero J, Bajo-Arenas JM. Renal angiomyolipoma during pregnancy: Review of the literature. Gynecol Obstet Invest. 2011;72(4):217–19. 10.1159/000329328
45. Le P, Garg A, Brandao G, Abu-Sanad A, Panasci L. Hormonal manipulation with letrozole in the treatment of metastatic malignant pecoma. Curr Oncol. 2014;21(3):e518–20. 10.3747/co.21.1849
46. Chan K, Chedgy E, Bent C, Turner K. Surveillance imaging for sporadic renal angiomyolipoma less than 40 mm: Lessons learnt and recommendations from the experience of a large district general hospital. Ann R Coll Surg Engl. 2018;100(6):480–4. 10.1308/rcsann.2018.0040
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Spectrum of Presentations and Management Strategies in Renal Angiomyolipoma. (2022). Journal of Kidney Cancer, 9(1), 42-47. https://doi.org/10.15586/jkcvhl.v9i1.221
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Spectrum of Presentations and Management Strategies in Renal Angiomyolipoma. (2022). Journal of Kidney Cancer, 9(1), 42-47. https://doi.org/10.15586/jkcvhl.v9i1.221