What extent of pancreatic resection do patients with MEN-1 require?

Context The surgical management of pancreatic endocrine tumors in patients with multiple endocrine neoplasia type 1 (MEN-1) is controversial and complicated by the fact that these tumors are frequently multifocal. The degree of tumor resection is determined by weighing the risk of malignancy or tumor recurrence against the risks of endocrine/exocrine insufficiency with complete gland removal. Methods A retrospective review was performed identifying 4 patients with MEN-1 and pancreatic endocrine tumors treated with pancreatic resection over a 2-year period at our institution. Results Mean age at operation was 35 years. Surgical approach was determined by size of tumor(s) and presence of multifocality. MRI and EUS were performed in all patients. While EUS identified a greater number of tumors when compared to MRI (median 5 versus 1), both studies grossly underestimated the total number of tumors found on final pathology. Three patients underwent laparoscopic total pancreatectomy for multifocal disease with diffuse pancreatic involvement, finding a median of 12 tumors. One patient underwent laparoscopic subtotal pancreatectomy for a presumed single pancreatic tail mass, but was found to have multifocal disease on final pathology consisting of 7 tumors. The average number of tumors found on final pathology was 13.5 with an average size of 2.6 cm. The median number of lymph nodes analyzed was 14. Diffuse, multifocal disease was present in all 4 patients. No major postoperative complications were observed. Conclusion In patients with MEN-1 and pancreatic endocrine tumors, preoperative workup underestimates extent of disease and total pancreatectomy should be considered for complete tumor removal.