Surgical management of pancreatic endocrine tumors

Pancreatic endocrine tumors (PETs) are uncommon but clinically challenging and fascinating tumors with an annual incidence of 1 per 100,000 people. PETs present as either functional pancreatic tumors or as nonfunctional pancreatic tumors. Functional tumors are commonly associated with a specific hormonal syndrome directly related to a hormone secreted by the tumor, such as Zollinger-Ellison syndrome or organic hyperinsulinism. Nonfunctional tumors do not secrete a hormone resulting in a pathologic syndrome of clinical symptoms. The natural history of PETs is highly variable. Small, benign neoplasms, such as 90% of all insulinomas, are readily curable by surgical resection; however, most other functional and all nonfunctional pancreatic tumors have a much less favorable prognosis. Patients with completely resected tumors generally have a good prognosis, and an aggressive surgical approach in patients with advanced disease may also prolong survival. Many features of the management of pancreatic endocrine tumors, such as timing and extent of resection, and the use of laparoscopic procedures, are currently under debate. This Review describes the current status of surgical treatment for pancreatic endocrine tumors, and discusses the new developments in this field.