An unusual case of pancreatic mass

A 61-year-old female affected by type 1 neurofibromatosis (NF-1) underwent abdominal imaging for nausea and hyporexia, finding a cephalo-pancreatic lesion with duodenal wall infiltration, and suspicions of pancreatic adenocarcinoma. An endoscopic exam revealed an occluding duodenal polypoid lesion of the periampullary area, with no clearly neoplastic pit-pattern. Biopsies were not diagnostic for malignancy. Contrast-enhanced Endoscopic Ultrasound confirmed a large lesion of pancreatic head, with duodenal wall involvement, and a fine-needle biopsy was performed. Histology revealed the presence of a well-differentiated Neuro-Endocrine Tumour (NET). The patient underwent duodenocefalopancreasectomy, with confirmation of a stenosing duodenal mass, and histological examination of surgical specimen finally deposed for a duodenal NET with pancreatic infiltration and lymph nodes involvement. Peri-ampullary NET, together with intestinal multiple gastrointestinal stromal tumour (GIST), are the most typical neoplasms in NF-1 patients. This case shows how such characteristic neoplasms must be taken into account during the evaluation of this specific population.