Abstract
BACKGROUND: Pancreatic neuroendocrine neoplasias (pNENs) are uncommon but fascinating tumors with an annual incidence of 1 per 100,000 people. pNENs present either as functional tumors, causing specific hormonal syndromes like Zollinger-Ellison syndrome (ZES) or organic hyperinsulinism, or as non-functional pancreatic tumors (NF-pNENs). The natural history of pNENs is highly variable. 90% 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. METHODS: A systematic review of the literature was performed to identify the current state of the art with regard to the key issues of surgery in pNEN G1/G2. RESULTS: This article provides a comprehensive review of the current literature addressing the current challenges in pNEN surgery. CONCLUSION: 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 rarely provides cure but often results in long-term survival.