肝脏血管周上皮样肿瘤7 例报告并文献复习

目的：探讨肝脏血管周上皮样肿瘤的临床病理学特征、诊断及鉴别诊断。方法：复习山西省肿瘤医院自2008年3 月到2015年3 月诊治的肝脏7 例标本，观察其组织病理学形态及免疫组化结果，并复习相关文献。结果：7 例患者平均发病年龄51.5岁（范围41～56岁），男性2 例，女性5 例。其中经典型2 例，肌细胞为主型3 例，脂肪细胞为主型2 例。免疫组织化学染色显示：7例病例HMB45均为阳性，6 例阳性SMA 及VIM ，5 例阳性MelanA，2 例结蛋白阳性，Ki-67平均阳性指数为1.3％（范围0～5％），TFE 3 均为阴性。结论：原发性肝脏血管上皮样肿瘤是一种肝脏罕见肿瘤，应提高对其认识和诊疗水平，术后应长期密切随访。免疫组织化学染色标记HMB45、MelanA和SMA 对于肝脏PEComa的诊断具有帮助作用。

Clinicopathological analysis of hepatic perivascular epithelioid cell carcinoma: a report on seven cases and literature review

Objective:To analyze the clinicopathological characteristics, diagnosis, and differential diagnosis of hepatic perivascu-lar epithelioid cell neoplasm (PEComa). Methods:The clinicopathological features of seven hepatic PEComa patients treated in Shanxi Cancer Hospital from March 2008to March 2015were studied with light microscopy and immunohistochemistry combined with litera-ture review. Results:The age range of the seven patients was41- 56years old (median: 51.5 years old). Two were males and five were females. Their histopathological subtypes were classic type ( 2/7), smooth muscle type ( 3/7), and fat type (2/7), respectively. Immunohis -tochemical study showed that HMB 45was positive in7 cases, SMA and VIM were expressed in6 cases, Melan A was positive in 5 cas-es, and Desmin was expressed in 2 cases. In addition, the mean proliferative index was 1.3% (range: 0%-5%), and TFE3 was negative in all cases. Conclusion:Hepatic PEComa is a rare primary liver tumor. The diagnosis and treatment of this disease should be intensi-fied, and long-term close follow-up is necessary. Positive result for HMB45, Melan A, and SMA are the key features of PEComa.