Autopsy and Case Reports
Autopsy and Case Reports
Clinical Case Report

Epithelioid angiomyolipoma with tumor thrombus in IVC and right atrium

Deepika Gupta; Vikarn Vishwajeet; Himanshu Pandey; Mahendra Singh; Binit Sureka; Poonam Elhence

Downloads: 2
Views: 1154


Epithelioid angiomyolipoma is an uncommon subtype of renal angiomyolipoma associated with potentially malignant behavior and is considered a distinct entity by the World Health Organization classification of renal tumors. We present a case of an epithelioid variant of angiomyolipoma with extension into the renal vein, inferior vena cava reaching up to the right atrium. Pre-operatively, a diagnosis of renal cell carcinoma was considered based on imaging findings. Intra-operatively due to extensive adhesions, surgical resection was not performed and only tissue sampling was performed for histopathology. Microscopic examination revealed short fascicles of spindle cells and perivascular epithelioid cells. A differential diagnosis of renal cell carcinoma with sarcomatoid differentiation was considered. The immunohistochemical profile showed tumor cells that express Melan-A and smooth muscle actin, while they were negative for pan-cytokeratin, PAX8, CK7, CD117 and CD34. Therefore a diagnosis of epithelioid angiomyolipoma was rendered. The presence of intravascular thrombi on radiological investigation and carcinoma-like growth pattern on light microscopy may compound an erroneous diagnosis of renal cell carcinoma. Hence, it is prudent for the urologist to consider differential diagnosis other than renal cell carcinoma when confronted with a renal neoplasm presenting with intravascular thrombi. In these cases, a core biopsy should be planned pre-operatively and diagnosis should be made with aid of appropriate immunohistochemical markers.


Kidney, Angiomyolipoma, Histology, Epithelioid Cells, Immunohistochemistry


Martignoni G, Pea M, Zampini C, et al. PEComas of the kidney and of the genitourinary tract.


Semin Diagn Pathol. 2015;32(2):140-59. []. [PMID:25804448]


Moch H, Humphrey PA, Ulbright TM, Reuter V.



WHO Classification of Tumours of the Urinary System and Male Genital Organs. Lyon, France: International Agency for Research on Cancer;2016


Aydin H, Magi-Galluzzi C, Lane BR, et al. Renal angiomyolipoma: clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association.



Am J Surg Pathol. 2009;33(2):289-97. []. [PMID:18852677]


Nelson CP, Sanda MG. Contemporary diagnosis and management of renal angiomyolipoma.



J Urol. 2002;168(4 Pt 1):1315-25. []. [PMID:12352384]


Fox C, Salami SS, Moreira DM, et al. Aggressive renal angiomyolipoma of the lipomatous variant with inferior vena cava thrombus: a case report and review of the literature.



Urol Case Rep. 2014;2(1):9-11. []. [PMID:26955534]


Riviere A, Bessede T, Patard JJ. Nephron sparing surgery for renal angiomyolipoma with inferior vena cava thrombus in tuberous sclerosis.



Case Rep Urol. 2014;2014:285613. []. [PMID:24778892]


Eble JN. Angiomyolipoma of kidney.



Semin Diagn Pathol. 1998;15(1):21-40. [PMID:9503504]


Vos N, Oyen R. Renal angiomyolipoma: the good, the bad, and the ugly.



J Belg Soc Radiol. 2018;102(1):41. []. [PMID:30039053]


Çalışkan S, Gümrükçü G, Özsoy E, Topaktas R, Öztürk . Renal angiomyolipoma.



Rev Assoc Med Bras. 2019;65(7):977-81. []. [PMID:31389508]


Park HK, Zhang S, Wong MK, Kim HL. Clinical presentation of epithelioid angiomyolipoma.



Int J Urol. 2007;14(1):21-5. []. [PMID:17199855]


Eble JN, Amin MB, Young RH. Epithelioid angiomyolipoma of the kidney: a report of five cases with a prominent and diagnostically confusing epithelioid smooth muscle component.



Am J Surg Pathol. 1997;21(10):1123-30. []. [PMID:9331283]


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. []. [PMID:20410812]


Nese N, Martignoni G, Fletcher CD, 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. []. [PMID:21263237]


Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F. PEComas: the past, the present and the future.



Virchows Arch. 2008;452(2):119-32. []. [PMID:18080139]


Kutcher R, Rosenblatt R, Mitsudo SM, Goldman M, Kogan S. Renal angiomyolipoma with sonographic demonstration of extension into the inferior vena cava.



Radiology. 1982;143(3):755-6. []. [PMID:7079506]


Rothenberg DM, Brandt TD, DʼCruz I. Computed tomography of renal angiomyolipoma presenting as right atrial mass.



J Comput Assist Tomogr. 1986;10(6):1054-6. []. [PMID:3537029]


Serrano Frago P, Del Agua Arias Camisón C, Jesus Gil Sanz M, et al. Controversies related to epithelioid variant of renal angiomyolipoma: a review of the literature.



Urology. 2006;67(4):846. []. [PMID:16600344]


Hung MS, Chang JH, Chang CP, Tai HL. Massive epithelioid angiomyolipoma of the kidney in a young girl.



Int J Urol. 2005;12(11):998-1000. []. [PMID:16351659]


Mojica WD, Jovanoska S, Bernacki EG. Epithelioid angiomyolipoma: appearance on fine-needle aspiration report of a case.



Diagn Cytopathol. 2000;23(3):192-5. [<192::AID-DC10>3.0.CO;2-V]. [PMID:10945908]


Wen J, Li HZ, Ji ZG, Mao QZ, Shi BB, Yan WG. Renal epithelioid angiomyolipoma without obvious local progress in 10 years: a case report and literature review.



Ir J Med Sci. 2011;180(2):557-60. []. [PMID:21042879]


Hohensee SE, La Rosa FG, Homer P, et al. Renal epithelioid angiomyolipoma with a negative premelanosome marker immunoprofile: a case report and review of the literature.



J Med Case Rep. 2013;7(1):118. []. [PMID:23628229]


Lin CY, Tsai CC, Kau HC, Yu WK, Kao SC, Liu CJ. HMB-45 negative angiomyolipoma of the orbit: a case report and review of the literature.



BMC Ophthalmol. 2016;16(1):8. []. [PMID:26754205]


























































Submitted date:

Accepted date:

Publication date:

5f4feddf0e8825e41d7dc2e3 autopsy Articles
Links & Downloads

Autops Case Rep

Share this page
Page Sections