Case Report
Copyright ©2006 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Gastroenterol. Oct 7, 2006; 12(37): 6070-6073
Published online Oct 7, 2006. doi: 10.3748/wjg.v12.i37.6070
Huge primitive neuroectodermal tumor of the pancreas: Report of a case and review of the literature
Thilo Welsch, Gunhild Mechtersheimer, Sebastian Aulmann, Sascha A Mueller, Markus W Buechler, Jan Schmidt, Peter Kienle
Thilo Welsch, Sascha A Mueller, Markus W Buechler, Jan Schmidt, Peter Kienle, Deptartment of Surgery, University of Heidelberg, INF 110, D-69120 Heidelberg, Germany
Gunhild Mechtersheimer, Sebastian Aulmann, Deptartment of Pathology, University of Heidelberg, INF 220, D-69120 Heidelberg, Germany
Correspondence to: Thilo Welsch, MD, Deptartment of Surgery, University of Heidelberg, Im Neuenheimer Feld 110, D-69120 Heidelberg, Germany.
Telephone: +49-6221-5639470 Fax: +49-6221-565781
Received: April 22, 2006
Revised: May 12, 2006
Accepted: June 16, 2006
Published online: October 7, 2006

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.

Keywords: Primitive neuroectodermal tumor, Pancreas, MIC2-protein, Ewing sarcoma, Abdominal mass