Seventy years of pheochromocytomas and paragangliomas in Argentina. The FRENAR database.

Autor: de Miguel VC; Hospital Italiano de Buenos Aires, Argentina. Electronic address: valeria.demiguel@hospitalitaliano.org.ar., Aparicio LS; Hospital Italiano de Buenos Aires, Argentina., Sansó G; Centro de Investigaciones Endocrinológicas Dr. César Bergadá, CONICET - FEI - División de Endocrinología, Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina., Paissan AL; Hospital Italiano de Buenos Aires, Argentina., Lupi SN; Hospital Ramos Mejía, Buenos Aires, Argentina., Belli SH; Instituto Alexander Fleming, Buenos Aires, Argentina., Tkatch J; Hospital Durand, Buenos Aires, Argentina., Marín MJ; Hospital Italiano de Buenos Aires, Argentina., Barontini MB; Centro de Investigaciones Endocrinológicas Dr. César Bergadá, CONICET - FEI - División de Endocrinología, Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina.
Jazyk: angličtina
Zdroj: Hipertension y riesgo vascular [Hipertens Riesgo Vasc] 2024 Jul-Sep; Vol. 41 (3), pp. 170-178. Date of Electronic Publication: 2024 Apr 30.
DOI: 10.1016/j.hipert.2024.04.001
Abstrakt: Pheochromocytomas and paragangliomas (PPGL) are neuroendocrine tumors characterized by the excessive production of catecholamines. This study aims to describe the clinical characteristics of PPGL cases in Argentina over recent decades. A multicenter retrospective cross-sectional analysis was carried out using a database comprising both pediatric and adult patients with confirmed PPGL diagnoses based on pathological reports. A cohort of 486 patients with PPGL was recruited. Women represent 58.4% of the patients, with a mean age of 38.3 years old at the time of diagnosis and 15.2% of the patients were under the age of 18. Hypertension, as well as classic signs and symptoms, were present in 80.9% of the patients. The adrenal incidentaloma, as a mode of presentation, increased in the last two decades rising from 3.9% (1953-2000) to 21.8% (2001-2022), p<0.001. Most tumors were located within the adrenal glands, accounting 83.0% of the cases, with bilateral occurrences noted in 20.0%. The median tumor size was 4.8cm. Local recurrence and metastases were observed in 10.9% and 12.2%. Out of 412 patients, 87.0% exhibited urinary excretion elevation of catecholamines and/or their metabolites. Furthermore, 148 patients, representing 30.4% of the study population, displayed a distinct genetic profile indicative of hereditary syndromes. The distribution of hereditary syndromes revealed that MEN2, VHL, and PGL4 constituted the most prevalent syndromes. This population-based study, spanning seven decades, offers valuable insights into the demographic and clinical characteristics of PPGL patients in Argentina.
(Copyright © 2024 SEH-LELHA. Publicado por Elsevier España, S.L.U. All rights reserved.)
Databáze: MEDLINE
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