Surgical strategies for nonfunctioning neuroendocrine pancreatic tumors and for other pancreatic neoplasms associated with multiple endocrine neoplasia type 1

Abstract

The most important step in the management of nonfunctioning neuroendocrine pancreatic tumors (NEPTs) is the determination of the primary tumor location and the tumor extent. In patients with localized, nonmetastatic disease, complete surgical resection of the primary tumor is the treatment of choice. For locally advanced, unresectable tumors, the surgical indication remains problematic. It is difficult to indicate palliative pancreatic resection due to the potential morbidity associated with debulking procedures and high recurrence rates. In patients with clinically nonfunctioning NEPTs in multiple endocrine neoplasia type 1 (MEN-1), the role of surgery is controversial. Based on the characteristic multifocality of the tumors, some have suggested that surgery should be limited to those larger than 2 cm in size (primary tumor size appears to correlate with metastatic potential). For insulinoma MEN-1 patients, it seems that subtotal distal pancreatectomy, preserving the spleen, combined with enucleation of any tumors identified in the pancreatic head, should be the standard operation. The role of surgery for MEN-1 Zollinger-Ellison syndrome (ZES) patients is debatable.