肝恶性血管周上皮样细胞肿瘤临床病理观察

Malignant perivascular epithelioid cell tumor of the liver: report of a case and review of literature

目的研究肝恶性血管周上皮样细胞肿瘤的临床病理学特点、诊断与鉴别诊断。方法收集1例肝恶性血管周上皮样细胞肿瘤的临床病理资料,并复习相关文献,总结其临床病理特点及免疫组化情况。结果本例组织形态学表现为肿瘤间质富于毛细血管、血窦。肿瘤细胞以上皮样细胞为主,大部分细胞的胞质嗜伊红色,小部分细胞透亮,部分细胞质内可见褐色色素颗粒。细胞异型显著,核分裂象易见,并可见病理性核分裂及凝固性坏死。PAS染色(+)。免疫组化:肿瘤细胞HMB45和melanoma(pan)弥漫强(+),部分细胞vimentin和SMA(+),Ki 67增殖指数70%。结论肝恶性血管周上皮样细胞肿瘤是一种少见的肿瘤,诊断时应注意与其他肿瘤相鉴别,尤其是肝细胞癌、恶性黑色素瘤以及转移性肾透明细胞癌。

Objective To study the clinicopathologic features of the hepatic malignant perivascular epithelioid cell tumor.Methods The clinicopathologic features were observed in 1 case of hepatic malignant perivascular epithelioid cell tumor; the literature was reviewed; Immunohistochemical features were analyzed. Results Morphologically,the stroma was rich in small blood vessels. The tumor was composed mainly of epithelioid cells with abundant eosinophilic to clear cytoplasm. Some tumor cells showed brown pigment granules. The tumor cells exhibited moderate to severe pleomorphism.The tumor showed high mitotic activity and necrosis. The tumor cells were positive for PAS. Immunohistochemically,the tumor cells demonstrated strong and diffuse positivity for HMB-45 and melanoma（ pan）,while vimentin and SMA were focally positive. About 70% of the neoplastic cells showed immunoreactivity for Ki67. Conclusions Perivascular epithelioid cell tumor is rare,and its differential diagnosis is necessary from other tumors,especially hepatocellular carcinoma,malignant melanoma and clear cell renal cell carcinoma.