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Multimodal Treatment of a Metastatic Pancreatic NeuroEndocrine Tumor in Von Hippel Lindau Disease


Introduction: Von Hippel-Lindau disease (VHL) is an inherited neoplastic syndrome: pancreatic lesions in 35-77% of cases, usually serous cystic adenoma (SCA), but 5-17% are non-functioning (NF) NeuroEndocrine tumors (NETs).

Aim(s): To report a case of a pancreatic NET in VHL, treated with surgery and targeted therapy

Materials and methods: In 2005 a 31-year-old woman with VHL diagnosis presented to our Clinic with sepsis/cholangitis. MRI: 4.0 cm solid-cystic mass of the pancreatic head, multiple pancreatic cysts (up to 3.0 cm). 111In-Octreoscan and 18F-FDG PET/CT both positive for the pancreatic mass. Normal serum tumor markers and GI hormones. Surgery: pylorus-preserving pancreatico-duodenectomy.

Conference: 14th Annual ENETSConcerence (2017)

Presenting Author:

Authors: Milanetto A, Liço V, Alaggio R, Pedrazzoli S, Pasquali C,

Keywords: Von Hippel-Lindau disease, NeuroEndocrine Tumor, Sunitinib,

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