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,

#4180 Non-functional alpha-cell hyperplasia with glucagonoma: A case report

Introduction: Alpha-cell hyperplasia(ACH) is a rare pancreatic endocrine condition. Clinically, 3 types have been described: functional ACH (nonglucagonoma hyperglucagonemic glucagonoma syndrome), reactive ACH (secondary to defective glucagon signalling) and non-functional ACH. Few cases of ACH and non-functional pancreatic neuroendocrine tumors (pNET) have been reported and its aetiology remains poorly understood.

Conference:

Presenting Author: Santos A

Authors: Cidade Rodrigues C, Santos A, Calheiros R, Santos S, Matos C,

Keywords: alpha-cell hyperplasia, diabetes, glucagon, pancreas, neuroendocrine tumor,

#4172 Hormonally characterised NF-PanNETs and their clinicopathological features

Introduction: PanNETs show pronounced hormonal heterogeneity. The expression of certain hormones may associate with functional syndromes, but also with histological and genetic features. However, detailed studies of hormonal patterns in PanNETs correlated to morphological and clinical data are lacking.

Conference:

Presenting Author: Moser E

Authors: Moser E, Ito A, Vogel L, Steiger K, Scheidhauer K,

Keywords: Neuroendocrine Tumor, hormonal expression, subtypes, outcome, transcriptional factors,

#3843 VIPoma presenting as chronic diarrhea in a 30-year-old Filipino female

Introduction: Pancreatic neuroendocrine tumors are rare neoplasms that arise in the endocrine tissues of the pancreas. They can secrete a variety of peptide hormones including insulin, gastrin, glucagon, and vasoactive intestinal peptide. Secretory tumors such as vasoactive intestinal polypeptides are rare neuroendocrine pancreatic neoplasms characterized by watery diarrhea, hypokalemia, and hypochlorhydria.

Conference:

Presenting Author: Edejer P

Authors: Edejer P, Mendoza R, Pascasio C, Lin T, Cornelio G,

Keywords: Pancreatic NET, Octreotide, DOTATATE, diarrhea,

#3734 Treatment outcomes in patients with localized and advanced glucagonomas – A case series

Introduction: Glucagonomas are rare functional pancreatic neuroendocrine tumors, which are often metastatic at diagnosis. Due to their low prevalence, the effectiveness of available treatment modalities remains unclear.

Conference:

Presenting Author: Armeni E

Authors: Porto J, Alexander B, Armeni E, Hesketh R, Mandair D,

Keywords: glucagonoma, treatment lines, progression-free survival, overall survival,