Huge primitive neuroectodermal tumor of the pancreas:Report of a case and review of the literature  

作　　者：Thilo Welsch Gunhild Mechtersheimer Sebastian Aulmann Sascha A Mueller Markus W Buechler Jan Schmidt Peter Kienle 

机构地区：[1]Deptartment of Surgery, University of Heidelberg, INF 110, D-69120 Heidelberg, Germany [2]Deptartment of Pathology, University of Heidelberg, INF 220, D-69120 Heidel- berg, Germany

出　　处：《World Journal of Gastroenterology》2006年第37期6070-6073,共4页世界胃肠病学杂志（英文版）

摘　　要：Primitive neuroectodermal tumor (PNET) of the pancreas is an extremely rare tumor that usually occurs in children or young adults. We report a case of a 33-year-old male patient with an 18 cm × 18 cm × 16 cm mass arising from the pancreatic body and tail with a one- day history of abdominal pain. Initial CT scan showed no signs of metastatic tumor spread. The tumor caused intrabdominal bleeding and the patient underwent primary tumor resection including partial gastrectomy, left pancreatic resection and splenectomy. Diagnosis of PNET was confi rmed by histology, immunohistochemistry and FISH analysis. All neoplastic cells were stained positive for MIC2-protein (CD99). Approximately one month after surgery, several liver metastases were observed and the patient underwent chemotherapy according to the Euro- Ewing protocol. Subsequent relaparotomy excluded any residual hepatic or extrahepatic abdominal metastases. Although PNET in the pancreas is an extremely rare entity, it should be considered in the diffential diagnosis of pancreatic masses, especially in young patients. This alarming case particularly illustrates that PNET in the pancreas although in an advanced stage can present with only a short history of mild symptoms.Primitive neuroectodermal tumor （PNET） of the pancreas is an extremely rare tumor that usually occurs in children or young adults. We report a case of a 33-year-old male patient with an 18 cm × 18 cm × 16 cm mass arising from the pancreatic body and tail with a one-day history of abdominal pain. Initial CT scan showed no signs of metastatic tumor spread. The tumor caused intrabdominal bleeding and the patient underwent primary tumor resection including partial gastrectomy, left pancreatic resection and splenectomy. Diagnosis of PNET was confirmed by histology, immunohistochemistry and FISH analysis. All neoplastic cells were stained positive for MIC2-protein （CD99）. Approximately one month after surgery, several liver metastases were observed and the patient underwent chemotherapy according to the Euro- Ewing protocol. Subsequent relaparotomy excluded any residual hepatic or extrahepatic abdominal metastases. Although PNET in the pancreas is an extremely rare entity, it should be considered in the diffential diagnosis of pancreatic masses, especially in young patients. This alarming case particularly illustrates that PNET in the pancreas although in an advanced stage can present with only a short history of mild symptoms.

关 键 词：Primitive neuroectodermal tumor PANCREAS MIC2-protein Ewing sarcoma Abdominal mass 

分 类 号：R735.9[医药卫生—肿瘤]