Abstract Library

#2986 Characterization of 142 Human Pancreatic Neuroendocrine Tumors: ATRX and DAXX Correlation with Clinical-Pathological Data

Introduction: Pancreatic neuroendocrine tumors (PNETs) are rare and heterogeneous neoplasms with a 10-year survival rate less than 30%. Up to 43% of them are driven by inactivating mutations in ATRX or DAXX. These are important players at chromatin remodeling, that when mutated prone cancer cells to initiate the alternative lengthening of telomeres (ALT). ALT phenotype correlates with in situ loss of expression of the proteins ATRX or DAXX and can be corroborated by telomere-FISH.

Conference: 17th Annual ENETSConcerence (2020)

Presenting Author:

Authors: Gaspar T, Pinheiro J, Canberk S, İnce �, Tokat F,

Keywords: pancreas, pancreatic neuroendocrine tumors, PNETs, ATRX, DAXX, ALT, mosaic,

#2885 Ectopic Neuroendocrine Carcinoma: An Occult Source of ACTH

Introduction: About 250 cases of ectopic ACTH-producing neuroendocrine carcinoma (ACTH-NEC) have been reported; however metastatic ACTH-NEC of unknown primary is very rare. Usually are characterized by having a large cell, small cell, poorly differentiated or anaplastic cell, and Ki-67 index between 40-90%.

Conference: 17th Annual ENETSConcerence (2020)

Presenting Author: Huertas Gnecco B

Authors: Huertas Gnecco B, González Devia D, Alvaréz Figueroa J, Aguirre Matallana D, Cañón Solano D,

Keywords: Ectopic ACTH production, Cushing’s syndrome, Neuroendocrine carcinoma, Paraneoplastic Cushing syndrome, Ectopic Cushing´s syndrome,

#2707 Investigating the Crosstalk between MEN1, p53 and Notch Reveals Biomarkers of Formation of Primary Pancreatic Neuroendocrine Tumors

Introduction: Pancreatic neuroendocrine tumors (PNETs) comprise a heterogeneous group of endocrine tumors. Previous studies showed that mutations of p53 are extremely rare; whereas genetic changes of MEN1 frequently occur and are correlated with poor prognosis in PNET. Still, their roles in tumorigenesis remain elusive.

Conference: 17th Annual ENETSConcerence (2020)

Presenting Author: Capodanno Y

Authors: Capodanno Y, Chen Y, Hiraoka N, Yokoyama A, Schrader J,

Keywords: MEN1, p53, Notch pathway, biomarkers, carcinogenesis, PNET,

#2245 Metastatic VIPOMA, Co-Secreting Insulin, with Complete Response to Lanreotide Combined with Capecitabine and Temozolamide

Introduction: VIPomas are rare neuroendocrine tumors (NETs) associated with vasoactive intestinal polypeptide (VIP) hypersecretion causing watery diarrhea, hypokalaemia and achlorhydria. They originate mostly in the pancreas and 60-80% are malignant. Hormonal co-secretion is rarely reported.

Conference: 15th Annual ENETSConcerence (2018)

Presenting Author: Marques B

Authors: Marques B, Martins R, Ribeiro J, Couto J, Ferrão H,

Keywords: vipoma, neuroendocrine, lanreotide, capecitabine, temozolamide,

#2198 PD-L1 Expression and Its Clinical Relevance in Neuroendocrine Tumors of the Lung

Introduction: Immune checkpoint regulation seems to be crucial for nonneuroendocrine lung cancer patients. However, little is known in lung neuroendocrine tumors.

Conference: 15th Annual ENETSConcerence (2018)

Presenting Author: Atsuko K

Authors: Kasajima A, Ishikawa Y, Iwata A, Steiger K, Oka N,

Keywords: Lung NEN, PD-L1, Prognosis,