Abstract Library

#1928 Pancreatic Involvement in VHL Disease: The Turin Experience

Introduction: Von Hippel-Lindau disease (VHL) is a rare heritable genetic syndrome. Its typical manifestations concern CNS but it may affect other organs such as the pancreas.

Conference: 14th Annual ENETSConcerence (2017)

Presenting Author: Cortegoso Valdivia P

Authors: Cortegoso Valdivia P, Venezia L, De Angelis C,

Keywords: VHL,

#1924 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).

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,

#1854 VHL Genotype Is Associated with Pancreatic Neuroendocrine Tumors (PNETs) Phenotype in Patients with Von Hippel-Lindau Disease (VHLD)

Introduction: 8-20% of patients with VHLD develop PNETs. However, there are no markers for PNETs progression in VHLD patients. The type of mutation in VHL gene is associated with clinical phenotype of VHLD.

Conference: 14th Annual ENETSConcerence (2017)

Presenting Author: Tirosh A

Authors: Tirosh A, Shell J, Keutgen X, Sadowski S, Green P,

Keywords: VHL, mutation, prognosis,

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

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.

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,

#40 Clinical and prognostic implications of the genetic diagnosis of hereditary NET syndromes in asymptomatic patients

Introduction: Neuroendocrine tumors (NETs) are rare neoplasms, heterogeneous, and with variable biological behavior. NETs can be sporadic or they can arise in complex endocrine hereditary syndromes such as Multiple Endocrine Neoplasias (MEN), Familial Paragangliomatosis (FPGL), Neurofibromatosis type 1 (NF1), von Hippel-Lindau Disease (VHL), Tuberous Sclerosis (TSC) and Carney Complex (CC). By performing genetic screening, patients with hereditary NETs can be identified before the development of tumors.

Conference: 7th Annual ENETSConcerence (2010)

Presenting Author:

Authors: Faggiano A, Ramundo V, Milone F, Severino R, Lombardi G,

Keywords: neuroendocrine tumor, genetic analysis, MEN1, MEN2, paragangliomatosis, genotype-phenotype correlation.,