Renal Epithelioid Angiomyolipoma with Aggresive Progression: A Case Report and Literature Review

Year 2021, Volume: 2 Issue: 2, 128 - 131, 05.05.2021

Emre Altıntaş , Ali Furkan Batur , Mehmet Kaynar , Murat Gül , Murat Akand Serdar Göktaş Zeliha Çelik , Özcan Kılıç

https://doi.org/10.47482/acmr.2021.26

Abstract

Epithelioid cellular morphology can be seen in clinically benign usual (or classic) angiomyolipoma (AML). Perivascular Epithelioid Cell Tumors (PEComa) are rarely seen as a variant of AML and usually benign in nature; however, they may have unpredictable pathological behavior. Here, we present a case of renal PEComa with malignant clinical progression and compare it with the current literature. A 56-year-old patient with a history of recurrent side pain present for about four months applied to our clinic. A hypodense mass was detected on the upper pole of the left kidney by ultrasonography. Computerized tomography showed an 8x4 cm mass originating from the upper pole of the left kidney and the adrenal gland, and was thought to invade the psoas muscle. The patient underwent a left transperitoneal radical nephrectomy. During the operation, vena cava inferior repair was required due to invasion and performed. Histopathologic examination revealed a PEComa. During the third month follow-up visit, a recurrent mass and lymph node enlargement were detected at the operation site. The mass was excised, and histopathology revealed a PEComa again. Considered as a rare variant of AML, PEComa is a tumor with the potential to exhibit malignant behavior. Although only a limited number of cases of renal PEComa have been reported; diagnosis, treatment, and follow-up are important due to their high potential for malignancy

Keywords

Renal Epithelioid Angiomyolipoma, PEComa, Angiomyolipoma.

References

• 1. Zhu J, Li H, Ding L, Cheng H. Imaging appearance of renal epithelioid angiomyolipoma: A case report and literature review. Medicine. 2018;97(1):e9563.
• 2. Hasan H, Howard AF, Alassiri AH, et al. PEComa of the terminal ileum mesentery as a secondary tumour in an adult survivor of embryonal rhabdomyosarcoma. Curr Oncol 2015;22:e383–6.
• 3. Martignoni G, Reuter VE, Fletcher CDM, World Health Organization (2016) Angiomyolipoma of World Health Organization classification of tumours of the urinary system and male genital organs. Lyon: IARC Press, pp 62–65
• 4. The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives.Sbaraglia M, Bellan E, Tos Dei A. Journal of The Italian Society of Anatomic Pathology and Diagnostic Cytopathology. Published: Nov 3, 2020. doi: 10.32074/1591-951X-213
• 5. Ameurtesse H, Chbani L, Bennani A, Toughrai I, Beggui N, Kamaoui I, Elfatemi H, Harmouch T, Amarti A. Primary perivascular epithelioid cell tumor of the liver: new case report and literature review. Diagn Pathol 2014; 9: 149.
• 6. Martignoni G, Pea M, Bonetti F, Zamboni G, Carbonara C, Longa L, Zancanaro C, Maran M, Brisigotti M, Mariuzzi GM. Carcinomalike monotypic epithelioid angiomyolipoma in patients without evidence of tuberous sclerosis: a clinicopathologic and genetic study. Am J Surg Pathol. 1998;22(6):663–72
• 7. Jayaprakash PG, Mathews S, Azariah MB, Babu G. Pure epitheliod perivascular epitheloid cell tumor (epitheliod angiomyolipoma) of kidney: case report and literature review. J Canc Res Therapeut. 2014;10(2):404–406.
• 8. Stone CH, Lee MW, Amin MB, et al. Renal angiomyolipoma: further immunophenotypic characterization of an expanding morphologic spectrum. Arch Pathol Lab Med 2001;125:751–758.
• 9. Ooi SM, Vivian JB and Cohen RJ: The use of the Ki-67 marker in the pathological diagnosis of the epithelioid variant of renal angiomyolipoma. Int Urol Nephrol 2009 41: 559-565.
• 10. Shi H, Cao Q, Li H, et al. Malignant perivascular epithelioid cell tumor of the kidney with rare pulmonary and ileum metastases. Int J Clin Exp Pathol 2014;7:6357–63.
• 11. Li W, Guo L, Bi X, Ma J, Zheng S. Immunohistochemistry of p53 and Ki-67 and p53 mutation analysis in renal epithelioid angiomyolipoma. Int J Clin Exp Pathol. 2015;8(8):9446–9451.
• 12. Brimo F, Robinson B, Guo C et al. 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.
• 13. Nese N, Martignoni G, Fletcher CD, et al. Pure spithelioid PEComas (socalled epithelioid angiomyolipoma) of the kidney: a clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. Am J Surg Pathol 2011; 35:161-76.
• 14. Cibas ES, Goss GA, Kulke MH, Demetri GD, Fletcher CD. Malignant epithelioid angiomyolipoma (‘sarcoma ex angiomyolipoma’) of the kidney: a case report and review of the literature. Am J Surg Pathol 2001; 25:121-6.