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Perivascular epithelial cell tumors (PEComas), firstly described by Bonetti in 1992, are a family of mesenchymal tumor composed of perivascular epithelioid cells having epithelioid or spindle morphology and exhibiting melanocytic and myogenic immunoreactivities. We herein described a 61-year-old woman who presented with epigastric pain. Preoperative imaging studies showed that 7-cm-sized mass was located in pancreatic head and body, and pancreaticoduodenectomy was performed. Histological findings showed that the tumor was composed of clear epithelioid cells with abundant glycogen granules, which grew in a nested and alveolar pattern around blood vessels. The tumor cells showed immunoreactivities for HMB-45 but did not express epithelial or endocrine markers. These histological features indicated those of PEComa. This report underlines that we should recognize PEComa as a preoperative differential diagnosis of pancreatic tumors.

作者:Yusuke, Mizuuchi;Kazuyoshi, Nishihara;Akifumi, Hayashi;Sadafumi, Tamiya;Satoshi, Toyoshima;Yoshinao, Oda;Toru, Nakano

来源:Surgical case reports 2016 年 2卷 1期

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作者:
Yusuke, Mizuuchi;Kazuyoshi, Nishihara;Akifumi, Hayashi;Sadafumi, Tamiya;Satoshi, Toyoshima;Yoshinao, Oda;Toru, Nakano
来源:
Surgical case reports 2016 年 2卷 1期
标签:
Alpha-smooth muscle actin HMB-45 Pancreatic neoplasm Perivascular epithelioid cell tumor
Perivascular epithelial cell tumors (PEComas), firstly described by Bonetti in 1992, are a family of mesenchymal tumor composed of perivascular epithelioid cells having epithelioid or spindle morphology and exhibiting melanocytic and myogenic immunoreactivities. We herein described a 61-year-old woman who presented with epigastric pain. Preoperative imaging studies showed that 7-cm-sized mass was located in pancreatic head and body, and pancreaticoduodenectomy was performed. Histological findings showed that the tumor was composed of clear epithelioid cells with abundant glycogen granules, which grew in a nested and alveolar pattern around blood vessels. The tumor cells showed immunoreactivities for HMB-45 but did not express epithelial or endocrine markers. These histological features indicated those of PEComa. This report underlines that we should recognize PEComa as a preoperative differential diagnosis of pancreatic tumors.