Management of the primary tumor in patients with metastatic pancreatic neuroendocrine tumor: a contemporary single-institution review

Background: Pancreatic nonfunctioning neuroendocrine tumors (PNFNETs) are an uncommon malignancy and often present with metastatic disease. There is a lack of information on the management of the primary tumor in patients who present with unresectable synchronous hepatic metastases. Methods: A retrospective review (2001-2008) of PNFNETs was conducted. Patients were divided into 3 groups: PNFNET without evidence of hepatic metastasis (group A), PNFNET with metastatic disease involving less than 50% of the liver (group B), and PNFNET with metastatic disease involving more than 50% of the liver (group C). Clinical data and outcomes were analyzed. Results: Thirty-five patients with PNFNET were identified (group A = 15, group B = 11, group C = 9). Resection of the pancreatic tumor was performed in 26 patients. With a mean follow-up period of 30 months, death from disease progression occurred in 1 patient in group A, none in group B, and in 7 in group C. Conclusions: In selected patients, resection of the primary pancreatic tumor even in the setting of unresectable but limited hepatic metastases may be indicated.