Long-Term Prognosis of Pancreatic Neuroendocrine Tumors in Von Hippel-Lindau Disease

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Introduction: Management of pancreatic neuroendocrine tumours (PNET) associated with von Hippel-Lindau (VHL) disease is challenging because of their malignant potential and poorly predictable prognosis.

Aim(s): To compare long-term outcome of resected VHL-PNET and sporadic PNET.

Materials and methods: All VHL patients (n=23) operated on for PNET in our centre were reviewed. Characteristics and recurrence-free survival (RFS) were compared to that of patients operated on for a sporadic PNET, matched for tumor size, stage and proliferation index.

Conference: 11th Annual ENETSConcerence (2014)

Presenting Author: de Mestier L

Authors: De Mestier L, Gaujoux S, Cros J, Hentic O, Vullierme M,

Keywords: pancreas, neuroendocrine tumors, von Hippel-Lindau disease, natural history, prognosis,