Published online Jan 21, 2015. doi: 10.3748/wjg.v21.i3.1036
Peer-review started: April 27, 2014
First decision: May 29, 2014
Revised: June 23, 2014
Accepted: July 24, 2014
Article in press: July 25, 2014
Published online: January 21, 2015
Paraganglioma is a rare tumor of paraganglia, derived from neural crest cells in sympathetic or parasympathetic ganglions. Primary paraganglioma originating from the pancreas is rare. We report two patients with paraganglioma in the head of the pancreas, in whom computed tomography showed a sharply marginated, hypervascular tumor with cystic areas. Significant intratumoral vessels and early contrast filling of the draining veins from the mass were not found. Although the pancreatic paraganglioma was located at the pancreatic head, the bile ducts often revealed no dilation, and sometimes the main pancreatic duct was mildly dilated. These findings are helpful in differentiating pancreatic paraganglioma from other pancreatic neoplasms. It is often difficult to distinguish between nonfunctional pancreatic paragangliomas and pancreatic endocrine tumors. In many reports,pancreatic paragangliomas show the retroperitoneal extension of a paraganglioma into the pancreas rather than a true pancreatic neoplasm. In surgical treatment, we could select simple excision of the tumor rather than radical surgery.
Core tip: Primary paraganglioma that arises in the pancreas is rare. We herein present two pathologically proven cases of pancreatic paraganglioma, retrospectively analyze their clinical and imaging features, and review the literature.