Sporadic pancreatic polypeptide secreting tumors (PPomas) of the pancreas.
BACKGROUND
Sporadic pancreatic neuroendocrine tumors, which predominantly secrete pancreatic polypeptide (PPoma), are rare and have not been associated with a clinical syndrome. A wider understanding of their pathological features and behavior is needed.


METHODS
Four PPoma patients who presented with nonspecific abdominal pain are described. Their diagnosis was established by the presence of an enhancing solitary pancreatic tumor on computed tomography (CT) and elevated fasting pancreatic polypeptide hormone levels. Patient 1 was treated with a pancreatoduodenectomy because of elevation of serum CEA level. Two of the cases underwent enucleation because of prolonged stable CT appearance. Patient 4 underwent distal pancreatectomy for a pancreatic neck tumor causing ductal obstruction and distal parenchymal atrophy.


RESULTS
All cases had benign histological features apart from patient 1 whose tumor demonstrated occasional mitotic activity. These tumors have not recurred after a median of 49 (range, 35-57) months. The protein expression in the tumor tissue was measured by SELDI-TOF MS and was different than the profile of pancreatic adenocarcinoma that was previously demonstrated in our laboratory. This may lead to future helpful diagnostic testing on fine needle aspirates.


CONCLUSIONS
Resection of sporadic PPomas presenting as a solitary well-defined mass with benign histological features results in good long-term survival.
University of Sydney, Department of Surgery, Royal North Shore Hospital, St Leonards, NSW 2065, Australia.
Samuel C L
SC
Kuo
Sivakumar
S
Gananadha
Christopher J
CJ
Scarlett
Anthony
A
Gill
Ross C
RC
Smith
D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology* / Q000601:surgery; D010191:Pancreatic Polypeptide / Q000378:metabolism*; D014057:Tomography, X-Ray Computed
D010191:Pancreatic Polypeptide