Operative management and long-term survival in patients with neuroendocrine tumors of the pancreas - Experience with 144 patients

Background and objective: To evaluate the outcome of patients with pancreatic endocrine tumors (PETs) in a tertiary referral centre. Methods: 144 patients with PETs that underwent surgery between 1987 and 2005 at our institution were retrospectively evaluated. The diagnosis of gastrinoma, insulinoma, vipoma and non-functioning PETs was based on clinical symptoms, biochemical tests and histopathology. Results: 144 patients were identified for this study, 20% with a MEN1-syndrome. 172 operations were performed, 122 initial operations and 50 reoperations. Enucleation of the tumor and distal pancreatic resections were the main type of operations. In 23 patients liver metastases were resected. After a median follow up of 67 months (range 1-339), 74 of 144 (51%) patients are still alive without evidence of disease. No patient with a benign tumor and no MEN1-patients died because of PETs. The 5, 10, and actuarial 20-year survival rate for patients with malignant tumors were 75%, 70% and 65%, respectively. The survival rate was significantly related to the type of tumor (benign vs. malignant: p = 0.0002), the patients age at time of initial operation (< 50 years vs. > 50 years: p = 0.0007), the genetic background of the tumor (sporadic vs. MEN1: p = 0.0312) and the development of metastases after the initial operation (none or lymph node metastases vs. distant metastases: p = 0.01). Conclusion: We show that an aggressive surgical approach leads to cure in patients with benign PETs. Although long-term cure can only be achieved in a proportion of patients with malignant PETs, significant long-term palliation can be achieved.