Abstract Library

#4614 Rare diagnosis of intrapancreatic accessory spleen mimicking PanNET

Introduction: A 44-year-old woman with hypertension and an autoimmune thyroid disease. With a positive family history of pancreatic and oesophageal cancer. Referred to our clinic for an incidental finding of a tumour in the tail of the pancreas on abdominal ultrasound by a surgeon.

Conference:

Presenting Author: Uhrík P

Authors: Uhrík P, Nosakova L, Vojtko M, Bánovčin P,

Keywords: fine needle biopsy, accessory spleen, Neuroendocrine tumour,

#4468 Prevalence of familial pheochromocytoma and paraganglioma syndromes – A large multicentre study

Introduction: Pheochromocytoma and paraganglioma (PPGLs) are rare tumours. Depending on the studied population, approximately 30% of PPGLs are caused by a germline pathogenic variant (PV).

Conference:

Presenting Author: Halperin R

Authors: Halperin R, Reznick-Levi G, Khalaileh A, Svirsky-Frayden R, Reish O,

Keywords: paraganglioma, pheochromocytoma, genetic evaluation, catecholamine, sdhb, vhl,

#4178 MEN1 syndrome across four generations

Introduction: MEN1 should be suspected in patients with an endocrinopathy of two of the three typically affected organs, or with an endocrinopathy of one of these organs and a first-degree relative affected by MEN1 syndrome. Still, a delay in diagnosis occurs in most cases.

Conference:

Presenting Author:

Authors: Ciobanu O, Martin S, Muha M, Fica S,

Keywords: MEN1 syndrome, large kindred, case series,

#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,

#3576 Patterns of family history of cancer and associated risk of developing neuroendocrine tumors: A cross-sectional study

Introduction: Except for known hereditary syndromes (mostly, multiple endocrine neoplasia type I and von Hippel Lindau), familial clustering of cancers in patients (pts) with neuroendocrine neoplasms (NEN) is scarcely reported.

Conference:

Presenting Author: Viapiana P

Authors: Viapiana P, Dias C, Cruz Formiga M, Simões Riechelmann R,

Keywords: neuroendocrine tumor, family history,