Objective: To analyze the surgical approach to multiple endocrine neoplasia type 1 (MEN 1)-related pancreatic endocrine tumors (PETs).
Methods: We reviewed selected publications and our personal experience with MEN 1-associated PETs to delineate their general characteristics, current practice and controversies, preoperative imaging and intraoperative assessment, and appropriate therapeutic strategies including radical surgical procedures.
Results: The penetrance of PETs in the setting of MEN 1 is similar to that of parathyroid tumors, even though hyperparathyroidism is usually the first manifestation of MEN 1 syndrome. In contrast with the sporadic counterparts, MEN 1-related PETs are characterized by an early onset, multiplicity of lesions, variable expression of the tumors, and propensity for malignant degeneration. Both the histologic type and the size of these tumors correlate with malignant potential.
Conclusion: The rationale for surgical considerations for these tumors is to curtail the malignant progression of the disease and to cure or aid in management of the associated biochemical syndromes. A surgical procedure is often the treatment of choice for PETs in patients with MEN 1. Monitoring of pancreatic peptides and use of diagnostic imaging allow an early pancreatic resection, in conjunction with prevention of metastatic PETs and improvement of long-term survival. Hepatic metastatic lesions can be successfully treated by surgical resection.