Giant Epithelioid Angiomyolipoma of Kidney - A Rare Case Report

Abstract

Renal angiomyolipoma is a benign mesenchymal neoplasm of kidney and composed of varied proportions of adipose tissue, smooth muscle cells- spindled or epithelioid and dysmorphic thickened or hyalinised blood vessels. Epithelioid angiomyolipoma is one of the rare variants of angiomyolipoma with high degree of complications, recurrence and metastasis. Whereas classic angiomyolipoma does not show metastasis and recurrence. Giant epithelioid angiomyolipoma means tumor size measuring greater than10 cm, very few cases have been reported in literature. Epithelioid angiomyolipoma of size greater than 20 cm is extremely rare. Here we report a rare case of giant epithelioid renal angiomyolipoma of size greater than 20 cm in a 21-year-old female, presented with complaints of right flank pain and hematuria. The patient underwent nephrectomy with probability of malignancy given in ultrasonography and specimen was sent for histopathological examination. Gross examination revealed enlarged kidney of size 22×18×10 cm and cut section shows grey white to yellow lesion measuring 21×17×10 cm almost occupying entire kidney. Microscopic examination revealed interlacing fascicles of spindle cells, lobules of adipocytes and thickened blood vessels. Foci of pleomorphic epithelioid cells with illdefined cell borders, pleomorphic vesicular nucleus and prominent nucleoli are noted. Keywords: Epithelioid, angiomyolipoma, mesenchymal, giant, kidney