Abstract Library

#3769 Adrenal neuroendocrine tumors in Oran – Epidemiology and survival

Introduction: Pheochromocytomas (PCC) are rare neuroendocrine tumors with a prevalence of 1/10000 in the general population. They are sporadic in 80% of cases, family forms are found in 10 to 20% of cases. PCCs represent 60% of multiple endocrine neoplasia type 2. They occur at any age with a peak between 30 and 50 years old. The clinical symptomatology dominated by arterial hypertension is linked to the hypersecretion of catecholamines. Metastases are common in the liver, bone or lung. The 5-year survival of malignant pheochromocytomas is less than 50%.

Conference:

Presenting Author:

Authors: Boumansour N, Khalifa S, Kehili H, Bengueddache A,

Keywords: adrenals, Neuroendocrine tumor, Pheochromocytoma, epidemiology, survival,

#3670 Response to peptide receptor radionuclide therapy in pheochromocytomas and paragangliomas – A systematic review and meta-analysis

Introduction: Peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE and 90Y-DOTATOC has shown efficacy in the metastatic setting of pheochromocytomas (PCCs) and paragangliomas (PGLs) where no standard therapies have been established.

Conference:

Presenting Author: Marretta A

Authors: Marretta A, Ottaiano A, Iervolino D, Bracigliano A, Clemente O,

Keywords: PRRT, Pheochromocytoma, Paraganglioma, Neuroendocrine Tumor, Meta-analysis,

#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,

#3478 Peptide Receptor Radionuclide Therapy with 177Lutethium or 90Yttrium- DOTA-TATE or –TOC in Malignant Pheochromocytomas (PCCs) and Paragangliomas (PGLs): Results from a single institutional retrospective analysis

Introduction: Malignant pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare tumors and limited systemic therapies are available so far.

Conference:

Presenting Author: Rubino M

Authors: Rubino M, Di Stasio G, Spada F, Rocca P, Laffi A,

Keywords: Pheochromocytoma, Paraganglioma, Peptide Receptor Radionuclide Therapy, 90Y, 177Lu,

#3257 Molecular and functional study of somatostatin receptors in pheochromocytomas and paragangliomas

Introduction: Most pheochromocytomas (PCC) and paragangliomas (PGL) are benign neuroendocrine tumors that overproduce catecholamines causing hypertension, arrhythmia, and stroke; however, 10 30% may present distant metastasis. About 40% are caused by germline mutations, which may be classified in 3 different clusters. PCC/PGL diagnosis involves blood/urine tests and imaging techniques, while their treatment often requires distinct pharmacological and surgical approaches. Thus, identification of new biomarkers for early diagnosis, and progression/response would help to avoid unnecessary tests and enable personalized medicine approaches.

Conference: 18th Annual ENETS Concerence (2021)

Presenting Author: Ibáñez-Costa A

Authors: Ibáñez-Costa A, Blázquez-Encinas R, Alors-Pérez E, Fuentes-Fayos A, Rivero-Cortés E,

Keywords: pheochromocytoma, paraganglioma, somatostatin, somatostatin receptor, neuroendocrine tumor,