肝脏血管周上皮样细胞肿瘤临床分析  

Clinical study of perivascular epithelioid cell tumor of liver: a literature review of 41 cases

作　　者：王刚[1] 高杰[1] 朱继业[1] 冷希圣[1] 胡志平[1] 

机构地区：[1]北京大学人民医院,北京100044

出　　处：《肝胆外科杂志》2015年第1期10-15,共6页Journal of Hepatobiliary Surgery

摘　　要：目的提高对罕见的肝脏原发性肿瘤——肝脏血管周上皮样细胞肿瘤（PEComa）的诊疗水平。方法采用文献检索收集1989~2013年间报道的包括北京大学人民医院1例在内资料齐全的原发性肝脏PEComa患者41例,总结其病例资料,对原发性肝脏PEComa的临床特点、影像学资料、病理特点、治疗及预后进行回顾性分析。结果肝脏PEComa无特异性临床表现,影像学特征不易识别,主要依靠术后病理确诊。手术是目前公认的主要治疗方法。41例患者中34例为良性,7例为恶性,恶性者预后较差。结论原发性肝脏PEComa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。Objective To improve the diagnosis and treatment of one type of rare primary liver tumor, perivascular epithelioid cell tumor （PEComa） of the liver. Methods A computer MEDLINE search found 41 histologically defined primary liver PEComa cases reported in the literature between 1989 and 2013 including one case from Peking University People＇s Hospital. The clinical features, imaging data, pathological features, therapeutic strategies and prognosis of the eases were summarized retrospectively. Result Liver PEComa has no specific clinical manifestations. Imaging features are uneasy to identify. Diagnosis mainly depends on postoperative pathological. Operation is the main treatment method recognized. In 41 patients, 34 cases were benign, and 7 were malignant with poor prognosis. Conclusion Liver PEComa is a type of rare primary liver tumor. The diagnosis and treatment of liver PEComa should be intensified. Long term close follow-up is necessary.

关 键 词：肝脏 血管周上皮样肿瘤 

分 类 号：R735.7[医药卫生—肿瘤]