Management of nonfunctioning pancreatic endocrine tumors in the context of multiple endocrine neoplasia type 1 syndrome

Abstract

The aim of our study is to present our experience in the surgical treatment of nonfunctioning pancreatic endocrine tumors (NFPETs) in patients with multiple endocrine neoplasia type 1 (MEN-1).

Patients and method: Between 1996 and 2006 a total of 11 patients with clinically confirmed MEN 1 syndrome were monitored in an annual screening program that included evaluation of the pancreas. Our policy was to use Computed Tomography (CT), Magnetic Resonance Imaging (MRI) and Endoscopic Ultrasound (EUS) in combination with biochemical screening in an effort to early diagnose and categorize the pancreatic involvement in MEN-1.

Results: NFPETs were identified in 4 female patients (36.4%). Diagnosis of NFPET was established 4.2 years later than that of MEN 1. The median tumor diameter at diagnosis was 2.2 cm (range 1.8-2.6 cm). All patients were treated by distal pancreatectomy. Diagnosis of NFPET was established in histological sections by staining with neuroendocrine tumor markers. Adjuvant therapy with streptozocin in combination with 5-fluorouracil was applied in two patients. After surgery the patients were followed up annually with clinical evaluation, biochemical tests and imaging studies.

Conclusions: Early detection of NFPETs in patients with MEN-1 syndrome can be accomplished by biochemical and radiological screening program. NFPETs should be removed when diagnosed, in order to achieve a timely and efficient prophylaxis against further tumor growth and malignant development.