Limaiem Faten, Korbi Skander, Lahmar Ahlem, Bouraoui Saâdia, Aloui Slim, Jedidi Souha, Miloudi Nizar, Mzabi-Regaya Sabeh
Department of Pathology, Mongi Slim Hospital, Sidi Daoued, La Marsa, Tunisia.
Acta Gastroenterol Belg. 2012 Dec;75(4):443-5.
Hepatic angiomyolipoma (HAML) is a rare, benign mesenchymal neoplasm composed of varying amounts of smooth muscle cells, adipose tissue, and vessels. Its morphological diversity often poses diagnostic problems. In this paper, the authors report a peculiar case of epithelioid HAML mimicking histologically hepatocellular carcinoma with focal areas resembling inflammatory pseudotumour. A 57 year-old male patient presented with abdominal pain and discomfort. Non enhanced CT scan demonstrated a heterogeneous hypodense mass located in segment II and IV of the liver. Hepatocellular carcinoma was suspected and the patient underwent left lobectomy. Histologically, the tumour was mainly composed of epithelioid cells arranged in trabeculae and sheets (50% of the tumour surface) admixed with mature fat cells (20%) and thick-walled blood vessels. Lymphocytic aggregates and clusters of foamy histiocytes were focally found in the stroma (30%). Most of the epithelioid tumour cells were immunoreactive to homatropine methylbromide 45 (HMB-45) and smooth muscle actin. Morphological pattern and immunophenotype were consistent with epithelioid HAML.
肝血管平滑肌脂肪瘤（HAML）是一种罕见的良性间叶性肿瘤，由不同数量的平滑肌细胞、脂肪组织和血管组成。其形态学多样性常常带来诊断难题。在本文中，作者报告了一例特殊的上皮样HAML病例，其组织学表现类似肝细胞癌，局部区域类似炎性假瘤。一名57岁男性患者出现腹痛和不适。非增强CT扫描显示肝脏Ⅱ段和Ⅳ段有一个不均匀的低密度肿块。怀疑为肝细胞癌，患者接受了左叶切除术。组织学上，肿瘤主要由排列成小梁状和片状的上皮样细胞组成（占肿瘤表面的50%），混有成熟脂肪细胞（20%）和厚壁血管。在间质中局部发现淋巴细胞聚集和泡沫状组织细胞簇（占30%）。大多数上皮样肿瘤细胞对甲基溴化后马托品45（HMB-45）和平滑肌肌动蛋白呈免疫反应性。形态学模式和免疫表型与上皮样HAML一致。
