肝脏非特异性血管周上皮样细胞肿瘤_附1例报告_张晓刚1_王林1_蒋依娜2_万真_.pdfVIP

网络出版时间：2014-01-17 16:38 网络出版地址：/kcms/doi/10.3969/j.issn.1673-4254.2014.01.011.html J South Med Univ, 2014, 34(1): 1-4 doi 10.3969/j.issn. 1673-4254.2014.01.01 ·1 · Case Report Hepatic perivascular epithelioid cell tumors-not otherwise specified: a case report 1 1 2 1 1 1 1 ZHANG Xiaogang , WANG Lin , JIANG Yina , WAN Zhen , LI Wenzhi , YAO Chunhe , GENG Zhimin , LÜ Yi1 1Department of Hepatobiliary Surgery, 2Department of Pathology, First Affiliated Hospital of Xian Jiaotong University College of Medicine, Xian 710061, China Abstract: Neoplasms of perivascular epithelioid cells (PEComas) are characterized by epithelioid to spindle cells with eosinophilic to clear cytoplasm, an intimate relationship with blood vessels, and coexpression of myoid and melanocytic immunohistochemical markers. While most reported hepatic PEComas, such as angiomyolipoma (AML), behave in a benign fashion, emerging PEComas cases without typical characteristics require further clarification. We report a case of primary hepatic perivascular epithelioid cell tumors-not otherwise specified (HPEComas-NOS) with untypical pathological and immunohistochemical features compared to those of the benign hepatic AML cases. HPEComas-NOS may represent a special type of PEComas classified as having malignant potential or at high risk of aggressive behavior, suggesting the need for further clarification of hepatic PEComas and long-term follow-up