Surgical experience with functioning pancreatic neuroendocrine tumors.

Pancreatic islet-cell tumors (ICTs) are rare malignancies usually recognized by specific clinical endocrinopathies. The purpose of this study is to evaluate our surgical experience with functioning pancreatic ICT in an academic referral center. Twenty patients (male:female 12:8) with a mean age of 53 years (range 26-82) underwent surgery for a functioning pancreatic ICT [gastrinoma (eight), multiple endocrine neoplasia (three), insulinoma (seven), glucagonoma (four), and VI-Poma (vasoactive intestinal peptide; one)] between June 1975 and March 2001. Signs and symptoms of hormonal excess were present in 95 per cent (19 of 20). One patient (glucagonoma) presented with obstructive jaundice and mild glucose intolerance. Elevated peptide levels were detected preoperatively in 65 per cent, including all patients with an insulinoma. Curative resections were attempted in 80 per cent including three procedures for insulinoma. Palliative procedures were performed in 20 per cent--all gastrinomas. One patient with an insulinoma had diffuse nesidioblastosis. Three patients (with gastrinoma, insulinoma, and glucagonoma) had lymph node-positive disease and three patients with gastrinoma had liver metastasis. The overall 30-day morbidity rate was 30 per cent and mortality rate 0 per cent. Symptomatic improvement was achieved in 90 per cent at a mean follow-up of 44 months. Two patients developed diabetes after a subtotal and a total pancreatectomy, respectively. Sixty-three per cent of patients who underwent an attempted curative resection are alive at a mean follow-up of 47 months (range 3-231) and all patients who underwent a palliative procedure are alive at a mean follow-up of 31 months (range 27-36). Functioning pancreatic ICTs are fascinating tumors that produce distinct clinical syndromes. Symptomatic improvement is accomplished in the majority of patients after surgery and short-term palliation is achieved in patients with nonresectable disease.