Abstract Library

#4651 Neuroendocrine hyperplasia of infancy (NEHI) vs. diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH): What is the difference?

Introduction: Neuroendocrine Hyperplasia of Infancy (NEHI) is a rare disease that belongs to the group of interstitial lung disease in children; characterised by hypoxemia, tachypnoea, and respiratory distress, often misdiagnosed as reactive airway disease or bronchiolitis. The diagnosis relies on histologic evaluation. We describe the clinical presentation and paraclinical characteristics of paediatric patients with NEHI.

Conference:

Presenting Author:

Authors: González Devia D, Restrepo Gualteros S, Ortiz Pérez M, Fernández Hernández B,

Keywords: Neuroendocrine Hyperplasia of Infancy, Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, child,

#4591 Efficacy of Trabectedin and Olaparib in homologous repair deficient neuroendocrine neoplasms – A subgroup analysis of the TOP-ART / PMO-1603 trial

Introduction: Genomic alterations resulting in homologous recombination deficiency (HRD) occur in a variety of cancers including neuroendocrine neoplasms (NEN). HRD-positive tumours are sensitive to PARP inhibitors such as olaparib. The DNA minor groove binder trabectedin leads to DNA double strand breaks and PARP activation. The combination of Trabectedin + Olaparib (TrO) may therefore have synergistic effects in HRD-positive tumours.

Conference:

Presenting Author: Apostolidis L

Authors: Apostolidis L, Ruebsam M, Teleanu M, Wagner S, Dorman K,

Keywords: Neuroendocrine Tumour, Neuroendocrine Carcinoma, targeted therapy, chemotherapy, HRD, olaparib, trabectedin, net, nec,

#4557 miR-1290 and miR-320c regulate cell proliferation and predict patient prognosis in neuroendocrine carcinomas

Introduction: The biology of neuroendocrine carcinomas (NECs) remains largely unknown, limiting the use of biomarkers for patient stratification and therapy response prediction in clinical practice.

Conference:

Presenting Author: Liverani C

Authors: Liverani C, Sgolacchia F, Bongiovanni A, Calabrese C, Vanni S,

Keywords: biomarker, miRNAs, neuroendocrine carcinoma,

#4417 Efficacy of immune checkpoint inhibitors (ICI) in patients with advanced pancreatic NETs displaying high TMB and MMR alterations following treatment with alkylating agents

Introduction: Alkylating chemotherapy (ALK) is frequently used in patients with PanNETs. It may favour grade progression and acquisition of a hypermutator phenotype associated with frequent alterations of the MMR genes, suggesting a potential benefit of ICI.

Conference:

Presenting Author:

Authors: de Mestier L, Apostolidis L, Koumarianou A, Hernando Cubero J, Riechelmann R,

Keywords: immune checkpoint inhibitor, immunotherapy, hypermutagenicity, alkylating agents, tumour mutational burden, mismatch repair,

#4364 Single agent axitinib in the management of patients with progressive pheochromocytoma and paraganglioma

Introduction: Radiolabelled therapy with meta-iodobenzguanine (MIBG) and chemotherapy with a combination of cyclophosphamide, vincristine, and dacarbazine can help manage patients with metastatic pheochromocytoma and paraganglioma (mPPGL). The phase II FIRSTMAPPP study supports the use of the tyrosine kinase inhibitor sunitinib in these malignancies, with median progression-free survival values of 8.9 versus 3.6 months and overall response rates of 31% versus 8% for sunitinib versus placebo. The median duration of sunitinib therapy was 11 months. Concurrent with this study, we launched a single-arm trial to examine the activity of a similar tyrosine kinase inhibitor, axitinib.

Conference:

Presenting Author: Del Rivero J

Authors: Del Rivero J, Fojo T, Edgerly M, Pacak K, Figg W,

Keywords: PPGL, pheochromocytoma, paraganglioma, axitinib, TKI, tyrosinase kinase inhibitors,