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,

#4020 Ovarian neuroendocrine tumor metastases can induce estrogen production in postmenopausal patients

Introduction: Neuroendocrine tumors (NET) are malignant neoplasms that can be associated with specific hormonal syndromes.

Conference:

Presenting Author: Mulders M

Authors: Mulders M, Hofland J, de Herder W, van Velthuysen M, Roes E,

Keywords: ovary, neuroendocrine, tumor, estradiol, postmenopausal, bleeding,

#3778 Biological behaviour and response to treatment of neuroendocrine ovarian metastases

Introduction: Neuroendocrine ovarian metastases (NOM) predominantly derive from midgut neuroendocrine tumors (NETs) and develop in about 25% of female patients. Little is known of the treatment response of NOM.

Conference:

Presenting Author:

Authors: Mulders M, de Lussanet de La Sablonière Q, van Velthuysen M, Hofland J, de Herder W,

Keywords: neuroendocrine, ovary, PRRT, SSA, oophorectomy, tumor, growth, rate, PFS, OS,

#3464 Digital quantification of somatostatin receptor subtype 2 immunostaining in pancreatic neuroendocrine tumors and GH-secreting adenoma: A validation study

Introduction: Somatostatin receptor subtype 2 (SST2) immunostaining (IHC) is routinely performed in neuroendocrine tumors (NET) and recommended in acromegaly guidelines. No univocal score of SST2 IHC has been validated yet.

Conference:

Presenting Author: Campana C

Authors: Campana C, van Koetsveld P, Iyer A, van Velthuysen M, van den Dungen E,

Keywords: SST2, digital image analysis, panNET, staining intensity, percentage of positive cell,

#2999 Aberrant Tryptophan Metabolism in Stromal Cells Is Associated with Mesenteric Fibrosis in Small Intestinal Neuroendocrine Tumours

Introduction: Increased levels of serotonin secretion are associated with mesenteric fibrosis (MF) in small intestinal neuroendocrine tumors (SI-NETs). However, only a proportion of patients with increased serotonin production will develop mesenteric fibrosis.

Conference: 17th Annual ENETSConcerence (2020)

Presenting Author: Blazevic A

Authors: Blazevic A, Iyer A, Van Velthuysen M, Hofland J, Franssen G,

Keywords: Small-intestinal neuroendocrine tumors, mesenteric fibrosis, serotonin, tryptophan metabolism, proteomics,