Symptomatic pancreatic polypeptide-secreting tumor of the distal pancreas (PPoma).
Our report describes a 46-yr-old woman who presented with watery diarrhea in the presence of multiple endocrine neoplasia type I (MEN I) syndrome. Of various potential pancreatic endocrine hormones, only serum levels of pancreatic polypeptide were elevated. Radiologic imaging failed to identify a pancreatic tumor; her diarrhea was therefore managed with subcutaneous administration of somatostatin. Three years later she developed gallstone pancreatitis with the subsequent development of a pancreatic pseudocyst. At exploration for drainage of the pseudocyst, intraoperative ultrasound identified a 6-mm tumor in the distal pancreas that was resected. Final pathology documented a pancreatic endocrine tumor with immunohistochemical staining demonstrating the presence of pancreatic polypeptide. The present case illustrates the symptomatology that may be associated with pancreatic polypeptide-secreting endocrine tumors of the pancreas.
Mortenson
Melinda
M
Department of Surgery, University of California Davis Medical Center, Sacramento, CA, USA.
Bold
Richard J
RJ
D002769:Cholelithiasis / Q000209:etiology; D003937:Diagnosis, Differential; D003967:Diarrhea / Q000209:etiology; D005260:Female; D006728:Hormones / Q000627:therapeutic use; D006801:Humans; D007150:Immunohistochemistry; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000150:complications / Q000473:pathology*; D010191:Pancreatic Polypeptide / Q000378:metabolism*; D010192:Pancreatic Pseudocyst / Q000209:etiology; D010195:Pancreatitis / Q000209:etiology; D013004:Somatostatin / Q000627:therapeutic use
D006728:Hormones; D013004:Somatostatin; D010191:Pancreatic Polypeptide