Abstract Library

#3859 Molecular landscape of splicing dysregulation in pheochromocytomas and paragangliomas

Introduction: Pheochromocytomas and paragangliomas (PPGL) are commonly benign neuroendocrine tumors (NETs). However, up to 25 % of patients develop metastases or aggressive behaviour. Genomic characterization current classifies PPGL into different genetic clusters, yet there are no biomarkers which could stratify patients based on their prognosis. The dysregulation of the splicing process has emerged as a novel feature, common in most cancers, and linked to a more aggressive phenotype in several tumors, including NETs, but this process has not been examined in detail in PPGL.

Conference:

Presenting Author:

Authors: Moreno Montilla M, Blázquez-Encinas R, Martínez Montes �, García Vioque V, Alors-Pérez E,

Keywords: pheochromocytoma, paraganglioma, splicing dysregulation, splicing machinery, pladienolide B,

#3853 Determining the resistance to somatostatin analogues in pheochromocytomas and paragangliomas

Introduction: Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine neoplasms (NENs) derived from chromaffin cells of the neural crest. Somatostatin analogues (SSAs) represent one of the pillars for NENs diagnosis/treatment due to the high density of somatostatin receptor (SSTs) in their membrane. However, although PPGLs share this characteristic, their response to SSAs is limited, being the reason underlying this unresponsiveness still unknown.

Conference:

Presenting Author:

Authors: García Vioque V, Blázquez-Encinas R, Moreno Montilla M, Mangili F, Martínez Montes �,

Keywords: pheochromocytoma, paraganglioma, neuroendocrine tumor, somatostatin analogues, treatment resistance,

#3493 Molecular characterization of the splicing machinery in pheochromocytomas and paragangliomas unveils a marked dysregulation with instructive potential

Introduction: Pheochromocytomas and paragangliomas(PPGL) are rare neuroendocrine neoplasms. Up to 25% will develop distant metastases or aggressive behaviour, but there is a lack of accurate risk markers able to stratify patients according to their potential outcome. Genomic characterization of PPGLs has identified 3 clusters: Pseudohypoxic-profile, MAPK-pathway alteration, and Wnt-pathway dysregulation. Alternative splicing dysregulation is emerging as a novel hallmark of cancer, due to its link to oncogenesis and aggressiveness in different neoplasms, including NENs.

Conference:

Presenting Author:

Authors: Moreno-Montilla M, Encinas Rey R, Martínez Montes A, García-Vioque V, Alors-Pérez E,

Keywords: pheochromocytoma, paraganglioma, metastasis, splicing dysregulation, splicing machinery,