Svajdler M, Bohus P, Goc V, Tkácová V
Oddelenie patológie, Fakultná nemocnica L. Pasteura Kosice, pracovisko Trieda SNP 1, Slovenská Republika.
Cesk Patol. 2007 Jan;43(1):18-22.
Perivascular epithelioid cell tumor (PEComa) is rare entity and has been described only recently. By immunohistochemistry and genetics it belongs to the family of tumours which comprises angiomyolipoma, clear cell "sugar" tumor of lung, lymphangioleiomyomatosis and clear cell myomelanotic tumor of ligamentum falciforme/teres hepatis. We describe an unusual case of hepatic PEComa arising in a 55-year-old woman with previous history of glioblastoma. Histologically the tumor grew in expansive way, and was composed of clear and eosinophilic epithelioid cels, without vascular or lipomatous component characteristic of angiomyolipoma. There was mild nuclear pleomorphism, sporadic mitotic activity and haemorrhage without necrosis. On immunohistochemistry, the tumor was HMB-45+50, Melan-A and smooth muscle actin positive. Tyrosinase, S-100 protein, cytokeratin coctail, EMA, vimentin, muscle specific actin, CD10, TTF-1, hepatocyte, desmin and cyclin D1 were negative. Sporadic nuclear p53 positivity was seen. The main differential diagnosis of hepatic PEComa includes clear cell variant of liver cell adenoma and hepatocellular carcinoma, metastases of various clear cell carcinomas and metastasis of malignant melanoma. In respect of uncertain biologic potential of PEComa, long term follow up is indicated.
血管周上皮样细胞肿瘤（PEComa）是一种罕见的肿瘤，直到最近才被描述。通过免疫组织化学和遗传学研究，它属于包括血管平滑肌脂肪瘤、肺透明细胞“糖”瘤、淋巴管平滑肌瘤病以及镰状韧带/肝圆韧带透明细胞肌黑色素瘤在内的肿瘤家族。我们报告一例罕见的肝脏PEComa病例，发生于一名55岁女性，既往有胶质母细胞瘤病史。组织学上，肿瘤呈膨胀性生长，由透明和嗜酸性上皮样细胞组成，无血管平滑肌脂肪瘤特有的血管或脂肪成分。有轻度核异型性、散在的有丝分裂活性和出血，但无坏死。免疫组织化学检查显示，肿瘤HMB-45、Melan-A及平滑肌肌动蛋白呈阳性。酪氨酸酶、S-100蛋白、细胞角蛋白鸡尾酒、EMA、波形蛋白、肌肉特异性肌动蛋白、CD10、TTF-1、肝细胞、结蛋白及细胞周期蛋白D1均为阴性。可见散在的核p53阳性。肝脏PEComa的主要鉴别诊断包括肝细胞腺瘤透明细胞变异型、肝细胞癌、各种透明细胞癌的转移以及恶性黑色素瘤转移。鉴于PEComa生物学行为不确定，需要长期随访。
