Periampulary and Pancreatic Neuroendocrine Neoplasms with Duodenal Stromal Gastrointestinal Tumors in Patients with Type 1 Neurofibromatosis: Two Case Reports


Introduction: We describe two cases of neuroendocrine tumors (NETs) in patients with neurofibromatosis type 1 (NF1) associated with gastrointestinal stromal tumors (GISTs).


Materials and methods: CASE 1 A 39-year-old female with NF1 and previous left surrenectomy for pheochromocytoma presented with a history of colicky postprandial pain, steatorrhea and weight loss. An MRI demonstrated the presence of a lesion of 15 mm into the Minor Papilla in pancreas divisum; EUS confirmed this finding (biopsies positive for NET). In September 2013, the patient underwent a pancreatoduodenectomy. Histology revealed bifocal Major and Minor Vater papilla NET G1 with Ki-67 2% (pT2 N0) associated with three small GISTs of the duodenum. The cells were immunostained positive to somatostatine. The patient is doing well. CASE 2: A 71-year-old man with NF1 and previous urological neoplasm, during follow-up CT-scan showed evidence of cephalic pancreatic lesion. EUS-biopsy positive for NET. In July 2012, the patient underwent a pancreatoduodenectomy. The histology revealed a G2 endocrine carcinoma, Ki-67 2%, two nodes metastases, and multiple small GISTs. Expression of somatostatine was strongly positive in tumor cells. At 17 months after the operation, the patient has had no disease recurrence.

Conference: 11th Annual ENETSConcerence (2014)

Presenting Author: Zerbi A

Authors: Ridolfi C, Gavazzi F, Spaggiari P, Carnaghi C, Lania A,

Keywords: pancreatic and ampullary neuroendocrine tumor, neurofibromatosis-1, GIST,

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