ANGIOMYOLIPOMA OF KIDNEY – A CASE REPORT

  • Dr. Usha Sarma Pathology Deptt, Gauhati Medical College, Guwahati, India
  • Dr. Rekha A Sangma Pathology Deptt, Gauhati Medical College, Guwahati, India
  • Dr. Ranjana Deka Pathology Deptt, Tezpur Medical College, Tezpur, India
Keywords: Nephrectomy, Renal angiomyolipoma, perivascular epithelioid cell, PEComa, HMB45, SMA

Abstract

Angiomyolipoma belongs to a family of neoplasms called perivascular epithelioid cell tumors (PEComas). The World Health Organization (WHO) defines PEComas as “mesenchymal tumors composed of histologically, ultrastructurally, and immunohistochemically distinctive perivascular epithelioid cells. Renal angiomyolipoma is an uncommon benign tumor characterized by a variable mixture of adipose tissue, smooth muscle, and thickened blood vessel and perivascular epithelioid cells also known as PEComa. Microscopically, the tumor shows mature adipose tissue, tortuous thick-walled blood vessels lacking elastic tissue lamina, and bundles of smooth muscle that seem to emanate from the vessel wall. A 55-year-old male patient presented with a huge abdominal painful lump in the left hypochondrium. Clinicoradiological evaluation suggests a mass lesion arising from the kidney. Nephrectomy was carried out. The cut section of the kidney showed multiple nodular yellow white and brown areas. Random sections from growth showed an admixture of Spindle cells & adipose tissue along with thick-walled blood vessels. Immunohistochemistry was carried out the result of which showed HMB 45 and Smooth Muscle Actin positive, but cytokeratin was negative. Considering the unilaterality, unifocal, larger size of the tumor and microscopic finding, and IHC result, the case was confirmed as a sporadic angiomyolipoma.

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References

1. Eble JN. Angiomyolipoma of the kidney. Semin Diagn Pathol 1998;15:21-40.
2. Apitz K: Die Gesch wülste und Geweb smissbildungen der Nierenrinde. II. Die mesenchymalen Neubildungen. Virchows Arch 1914;311:306-27.
3. Bonetti F, Pea M, Martignoni G, Zamboni G, Manfrin E, Colombari R, et al. The perivascular epithelioid cell and related lesions. Adv Anat Pathol 1997;4:343-58.
4. Steiner MS, Goldman SM, Fishman EK, Marshall FF. The natural history of renal angiomyolipoma. J Urol 1993; 150: 1782- 86.
5. Yang L, Feng XL, Shen S, Shan L, Zhang HF, Liu XY, et al. Clinicopathological analysis of 156 patients with angiomyolipoma originating from different organs. Oncol Lett 2012;3:586–90.
6. Pea M, Bonetti F, Zamboni G, Martignoni G, Riva M, Colombari R, et al.Melanocyte-marker-HMB-45 is regularly expressed in angiomyolipoma of the kidney. Pathology (1991) 23;185–88.
7. Ghada El Sayed Esheba & Noha El Sayed Esheba. Angiomyolipoma of the kidney: Clinicopathological and immunohistochemical study. Journal of the Egyptian National Cancer Institute. 2013; 25:125–34.
8. 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; 21:1123-30.
9. Zamecnik M, Majercik M, Gomolcak P. Renal. angiomyolipoma resembling gastrointestinal stromal tumor with skenoid fibers. Ann Diagn Pathol 1999; 3:88-91.
10. Nese N, Martignoni G, Fletcher CD, Gupta R, Pan CC, Kim H, et al. Renal perivascular epithelioid cell tumors [(PEComa), so-called epithelioid angiomyolipoma (EAML)]: analysis of 61 cases including 44 with pure/predominant epithelioid (P-PEComa) morphology and parameters associated with malignant outcome. Lab Invest 2009; 89 (Suppl 1):186A.
How to Cite
1.
Sarma U, Sangma RA, Deka R. ANGIOMYOLIPOMA OF KIDNEY – A CASE REPORT. Med. res. chronicles [Internet]. 2015May12 [cited 2024Nov.22];2(3):311-4. Available from: https://medrech.com/index.php/medrech/article/view/86
Section
Case Report