Ps-P10-7: Seventy Years of Pheochromocytomas and Paragangliomas in Argentina

Abstract

In Argentina, data regarding Pheochromocytomas and Paragangliomas (PPGLs) are scarse.Objective:to describe demographic, biochemical and genetic characteristics of children and adults with confirmed PPGls.Design and method:Observational multicenter study of recruited participants surveyed from a retrospective cross sectional database from january 1953 until January 2022.Results:We included 486 patients (58.4% women) from 93 recruiting centers, mean age at diagnosis 38.3, 393 (80.9%) hypertensives, baseline office systolic/diastolic blood pressure 157.8/96.5 mmHg. Of all tumors, 382 (79.6%) were Pheochromocytomas, 67 (13.8%) paragangliomas and 11 (2.3%) mixed. The most prevalent cathecolamin secretion type was noradrenergic (35%), followed by adrenergic (30.6%) and mixed (20.3%). Local recurrence and mestastasis occured in 10% of subjects. The most common symptoms were headache (57%), sweating (57.5%) and palpitations (53.4%). In 147 patients with a genetic syndrome, we found 44 MEN2A, 7 MEN2B, 1 MEN1, 42 Von Hippel Lindau, 11 neurofibromatosis type 1, 32 PGL4, 6 PGL1, 2 MAX, 1 TMEM127, and 1 mosaic EPAS1/HIF2A. We found the genetic variant types in 102 patients.Conclusions:Our results are consistent with other publications from foreign databases. PPGls are rarely diagnosed in Argentina, and the genetic profile is lacking in three fourths of patients.