Epithelioid Angiomyolipoma of Liver with an Inflammatory Component: A Case Report

Angiomyolipomas (AMLs) are benign mesenchymal tumors seen in kidneys in association with tuberous sclerosis. They are uncommon in liver. Angiomyolipomas of liver show great histological diversity and various types and patterns are described. Among them, epithelioid and inflammatory angiomyolipomas are rare. We report a case of epithelioid angiomyolipoma of Liver with an inflammatory component. 1. Introduction Angiomyolipomas are benign mesenchymal tumors seen in the kidney either sporadically or in association with tuberous sclerosis [1]. They belong to the perivascular epithelioid cell tumor family and coexpress melanocytic and smooth muscle cell markers [2]. Hepatic angiomyolipomas were first described by Ishak in 1976 [3]. Only 200 cases have been reported in the English literature so far [4]. They are seen most commonly in adult females [5] and are located in the right lobe of liver [6]. These lesions are difficult to diagnose by imaging studies, especially when the fat component is scant or absent [7]. Because of their epithelioid morphology, they may be mistaken for hepatocellular carcinoma [8]. Presence of inflammatory cells in epithelioid angiomyolipoma is rare, and it may resemble an inflammatory myofibroblastic tumor [9]. Immunohistochemistry is useful for diagnosis as the smooth muscle cells are positive for HMB45 and SMA [10]. 2. Case Report A 50-year-old gentleman came to the casualty with a history of fever, vomiting, and abdominal pain. He was a nonsmoker and nonalcoholic with no history of diabetes mellitus or hypertension. The patient did not have any features of tuberous sclerosis complex. An ultrasonogram showed a mass in the liver. CT showed a large mass in the left lobe of liver suggestive of hepatocellular carcinoma or giant angioma (Figure 1). The patient underwent lateral segmentectomy of liver. Figure 1: CT liver showing a mass lesion. We received segmentectomy specimen of liver which weighed 650 grams and measured ？cms. Surface was bosselated (Figure 2). Cut section showed a well-encapsulated cystic mass measuring 8？cms in diameter. Cut surface showed necrotic and hemorrhagic appearance with grey white firm areas in the periphery (Figure 3). Figure 2: liver with a cystic lesion. Figure 3: c/s of lesion—brownish, haemorrhagic, and necrotic. Microscopy showed a neoplasm composed of epithelioid cells, thick-walled blood vessels, and adipocytes. The epithelioid cells had granular pink or vacuolated cytoplasm and vesicular oval/round nuclei, some showing prominent nucleoli. There was pigment in some of the cells. Thick-walled blood