Abstract Library

#3848 LITESPARK-015 cohort B1 – A phase 2 open-label study of Belzutifan (a HIF-2α inhibitor) monotherapy in patients with von Hippel-Lindau (VHL) disease–associated tumors

Introduction: Patients (pts) with von Hippel-Lindau (VHL) disease need novel targeted therapies. Hypoxia-inducible factor 2α (HIF-2α) is a key oncogenic driver in VHL disease. Belzutifan (MK-6482), a HIF-2α inhibitor, has shown antitumor activity in pts with advanced renal cell carcinoma (RCC) and pancreatic neuroendocrine tumors (pNETs); more data are needed in VHL disease–associated localized tumors.

Conference:

Presenting Author:

Authors: Walter T, Gong K, Nakamura E, Iliopoulos O, Jimenez C,

Keywords: von Hippel-Lindau disease, belzutifan, MK-6482,

#3790 Pancreatic neuroendocrine tumors (pNETs) in French VHL mutation carriers – A nationwide retrospective study with genotype-phenotype correlations

Introduction: Von Hippel-Lindau disease (VHL) is a rare inheritable syndrome predisposing to pancreatic neuroendocrine tumors (pNETs). The natural history of VHL-related pNETs is ill-known.

Conference:

Presenting Author:

Authors: Muller M, Hammel P, Sauvanet A, Couvelard A, Vullierme M,

Keywords: hereditary neoplastic syndrome, von Hippel-Lindau disease, VHL gene, pancreatic neuroendocrine tumor,

#3748 Association of MINEN with GIST on neurofibromatosis – A case report

Introduction: Although most digestive neuroendocrine tumors (NETs) are sporadic, around 5% of NETs occur in a context of hereditary predisposition, mainly type 1 multiple endocrine neoplasia (NEM1) and Von Hippel-Lindau disease (VHL).

Conference:

Presenting Author:

Authors: Samia K, Fatima H, Nabil B, Benali T, Aisha B,

Keywords: NEN, GIST, MINEN, DOG1,

#3585 Belzutifan, an oral hypoxia-inducible factor 2α inhibitor, for Von Hippel-Lindau disease–associated pancreatic neuroendocrine tumors

Introduction: Constitutive activation of hypoxia-inducible factor 2α (HIF-2α) drives tumor growth, which include pancreatic lesions (serous cystadenomas and pancreatic neuroendocrine tumors [pNETs]), in Von Hippel-Lindau (VHL) disease. Belzutifan, an oral HIF-2α inhibitor, is approved for patients with VHL disease based on the results of this ongoing open-label phase 2 study (NCT03401788).

Conference:

Presenting Author:

Authors: Else T, Jonasch E, Iliopoulos O, Rathmell W, Narayan V,

Keywords: VHL, pancreatic neuroendocrine tumor, pNET, HIF-2α, belzutifan,

#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,