AUTHOR=Araujo-Castro Marta , García Sanz Iñigo , Mínguez Ojeda César , Hanzu Felicia , Mora Mireia , Vicente Almudena , Blanco Carrera Concepción , de Miguel Novoa Paz , López García María del Carmen , Lamas Cristina , Manjón-Miguélez Laura , del Castillo Tous María , Rodríguez de Vera Pablo , Barahona San Millán Rebeca , Recasens Mónica , Tomé Fernández-Ladreda Mariana , Valdés Nuria , Gracia Gimeno Paola , Robles Lazaro Cristina , Michalopoulou Theodora , Álvarez Escolá Cristina , García Centeno Rogelio , Barca-Tierno Verónica , Herrera-Martínez Aura D. , Calatayud María TITLE=Local recurrence and metastatic disease in pheochromocytomas and sympathetic paragangliomas JOURNAL=Frontiers in Endocrinology VOLUME=14 YEAR=2023 URL=https://www.frontiersin.org/journals/endocrinology/articles/10.3389/fendo.2023.1279828 DOI=10.3389/fendo.2023.1279828 ISSN=1664-2392 ABSTRACT=Purpose

To evaluate the rate of recurrence among patients with pheochromocytomas and sympathetic paragangliomas (PGLs; together PPGLs) and to identify predictors of recurrence (local recurrence and/or metastatic disease).

Methods

This retrospective multicenter study included information of 303 patients with PPGLs in follow-up in 19 Spanish tertiary hospitals. Recurrent disease was defined by the development of local recurrence and/or metastatic disease after initial complete surgical resection.

Results

A total of 303 patients with PPGLs that underwent 311 resections were included (288 pheochromocytomas and 15 sympathetic PGLs). After a median follow-up of 4.8 years (range 1-19), 24 patients (7.9%) had recurrent disease (3 local recurrence, 17 metastatic disease and 4 local recurrence followed by metastatic disease). The median time from the diagnosis of the PPGL to the recurrence was of 11.2 months (range 0.5-174) and recurrent disease cases distributed uniformly during the follow-up period. The presence of a pathogenic variant in SDHB gene (hazard ratio [HR] 13.3, 95% CI 4.20-41.92), higher urinary normetanephrine levels (HR 1.02 per each increase in standard deviation, 95% CI 1.01-1.03) and a larger tumor size (HR 1.01 per each increase in mm, 95% CI 1.00-1.02) were independently associated with disease recurrence.

Conclusion

The recurrence of PPGLs occurred more frequently in patients with SDHB mutations, with larger tumors and with higher urinary normetanephrine levels. Since PPGL recurrence may occur at any time after the initial PPGL diagnosis is performed, we recommend performing a strict follow-up in all patients with PPGLs, especially in those patients with a higher risk of recurrent disease.