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

VernacularTitle:肝脏血管周上皮样肿瘤7例报告并文献复习
Author: Yuanyuan ZHAO ; Enwei XU ; Jing LI
Publication Type:Journal Article
Keywords: liver neoplasms; perivascular epithelioid cell tumor; immunohistochemistry
From: Chinese Journal of Clinical Oncology 2015;42(22):1100-1102
CountryChina
Language:Chinese
Abstract: 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 2008 to March 2015 were studied with light microscopy and immunohistochemistry combined with litera-ture review. Results:The age range of the seven patients was 41-56 years 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 HMB45 was positive in 7 cases, SMA and VIM were expressed in 6 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.