Outcomes of resected nonfunctional pancreatic neuroendocrine tumors: Do size and symptoms matter?

Abstract

Nonfunctional pancreatic neuroendocrine tumors (NF-PNETs) are rare tumors with highly variable outcome. Current guidelines recommend surveillance for small tumors (≤ 2 cm), but a scientific basis for such recommendation is scarce. Patients who underwent surgery for NF-PNET during 2001-2013 were identified from a prospectively maintained database and reviewed retrospectively. Fifty-eight patients that had undergone an operative procedure for NF-PNET were identified. Forty-one patients (71%) were symptomatic. Median size of the tumor was 2.5 cm (range 0.9-12.0 cm). WHO 2010 grade was predictive of both overall- and disease-free survival (P < .001), whereas size alone was not. Twenty-four patients had a small NF-PNET (≤ 2 cm), of whom 16 were symptomatic and 8 asymptomatic. Seven patients with small symptomatic NF-PNETs showed signs of malignant behavior: 4 had lymph node metastases, 1 had liver metastases before surgery, 3 developed liver metastases, and 3 died of the disease. All 7 patients had either bile duct or pancreatic duct obstruction or both on preoperative imaging. On the contrary, patients with small asymptomatic NF-PNETs did not develop distant metastases nor died of disease. The 2010 grading system from the World Health Organization can be used to predict survival. Symptomatic small NF-PNETs that caused bile and/or pancreatic duct obstruction had poor outcome. In contrast, asymptomatic small NF-PNETs seem to have benign course, and are candidates for surveillance.