Angiomyolipoma is apparently part of family of neoplasms that derive from perivascular epitheloid cells. It is a rare mesenchymal tumor, usually found in the kidney. Extrarenal angiomyolipoma is uncommon and the most common extrarenal site is the liver. Only two cases of adrenal angiomyolipoma are reported in English literature. The authors wish to add one more case to the world literature. Because of its large size and symptomatic presentation this extremely rare tumor merits documentation. Case Report A 45 year old female presented with epigastric discomfort off and on. Upper G.I Endosocopy was normal. Sonography for hepatobiliary system was normal but revealed a well defined 15x12cm mass in the retroperitoneum (incidentaloma). CECT abdomen further defined the mass as of left adrenal origin and a possibility of adrenocortical tumour (Fig - I). Laboratory investigations i.e Serum catecholamine, cortisol and urinary VMA were within normal limits. Exploratory laparotomy revealed 15x12x10cm mass, firm in consistency, quite separate from left kidney with no definable left adrenal gland. On cut section mass was grey-white and non-homogeneous in texture (Fig – II). Histopathological examination revealed mature fat cells, smooth muscle fibres and thin walled blood vessels with peripherally compressed adrenal cortical tissue suggestive of angiomyolipoma of adrenal (Fig – III & IV). The patient made uneventful recovery and was normal at 18 months follow up.
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