Sporadic Pancreatic Endocrine Tumors

Abstract

Pancreatic endocrine neoplasias (pNENs) are uncommon but fascinating tumors with an annual incidence of 1 per 100,000 people. pNENs present as either functional tumors, causing specific hormonal syndromes like Zollinger-Ellison syndrome (ZES) or organic hyperinsulinism, or as pancreatic endocrine nonfunctional tumors (NF-pNENs). The natural history of pNENs is highly variable. Ninety percent of all insulinomas or small NF-pNENs are readily curable by surgical resection. Most other functional and late detected NF-pNENs have a less favorable chance for cure. Patients with completely resected tumors generally have a good prognosis, and an aggressive surgical approach combined with conservative treatment options in patients with advanced disease often results in long-term survival.