Abstract Library

#4353 Familial inactivating glucagon receptor mutation resulting in pancreatic neuroendocrine tumours with metastatic potential, somatic MEN1 mutations, and a heterozygous phenotype

Introduction: Homozygous pathogenic glucagon receptor (GCGR) mutations cause a syndrome with pancreatic glucagon cell hyperplasia and neoplasia (GCHN) associated with Mahvash disease. This is an exceptionally rare autosomal recessive hereditary pancreatic neuroendocrine tumour (panNET) syndrome, with approximately ten cases documented in the literature.

Conference:

Presenting Author: Kuiper J

Authors: Kuiper J, de Herder W, Brahim Y, van Velthuysen M, Brosens L,

Keywords: glucagon receptor mutation, mahvash disease, glucagon cell hyperplasia and neoplasia, pancreatic neuroendocrine tumour, MEN1,

#4156 Status of the ongoing SORENTO clinical trial: Assessing efficacy and safety of high-exposure octreotide subcutaneous depot in patients with GEP-NET

Introduction: Somatostatin receptor ligands (SRLs) are first-line standard of care therapies for gastroenteropancreatic neuroendocrine tumors (GEP-NET). Retrospective data suggest higher-dose SRLs may benefit patients with GEP-NET.

Conference:

Presenting Author: Singh S

Authors: Singh S, Capdevila J, Ang Chan J, de Herder W, Grozinsky-Glasberg S,

Keywords: CAM2029, GEP-NET, high plasma exposure, octreotide, randomised active-controlled, somatostatin receptor ligands, SORENTO,

#4155 [177Lu]Lu-DOTA-TATE in newly diagnosed patients with advanced grade 2 and grade 3, well-differentiated gastroenteropancreatic neuroendocrine tumors: Primary analysis of the phase 3 randomised NETTER-2 study

Introduction: There is no universally accepted first line (1L) therapy for higher grade, well-differentiated gastroenteropancreatic neuroendocrine tumors (GEP-NETs).

Conference:

Presenting Author: de Herder W

Authors: de Herder W, Halperin D, Myrehaug S, Herrmann K, Pavel M,

Keywords: [177Lu]Lu-DOTA-TATE, Lutathera, Gastroenteropancreatic Neuroendocrine Tumor, NETTER-2,

#4129 Validation of a radiomics model to predict symptoms complications from small intestinal NET mesenteric metastases – Preliminary report

Introduction: The development of mesenteric metastases and associated mesenteric fibrosis in small intestinal neuroendocrine tumors (SI-NET) can cause significant complications and have implications in patients’ survival. CT is suboptimal for accurate assessment of the degree of fibrosis and prediction of complications. A better model is needed, therefore, in order to select patients who may benefit from a prophylactic surgical approach in the mesentery. Recently the promising role of a radiomics model was shown as a predictive tool for development of complications of mesenteric metastases/fibrosis in 68 patients.

Conference:

Presenting Author:

Authors: von Stempel C, Blazevic A, Starmans M, d' Afflitto M, de Herder W,

Keywords: small intestinal neuroendocrine tumor, mesenteric fibrosis, complications, radiomics,

#4085 Disease-specific mortality in a single-center cohort of 427 patients with carcinoid syndrome

Introduction: The carcinoid syndrome (CS), the most prevalent hormonal syndrome in patients with a neuroendocrine tumor (NET), is associated with impaired survival. Due to the biological behaviour of NET and overall improvement in survival rates, patients with CS may not succumb to their disease. However, the contribution of NET-specific death in these patients is currently unknown.

Conference:

Presenting Author: Maas C

Authors: Maas C, Mulders M, Feelders R, de Herder W, Hofland J,

Keywords: carcinoid syndrome, neuroendocrine tumor, prognosis, survival analysis,