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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.;

Local recurrence and metastatic disease in pheochromocytomas and sympathetic paragangliomas

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  • Media type: E-Article
  • Title: Local recurrence and metastatic disease in pheochromocytomas and sympathetic paragangliomas
  • Contributor: 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.;
  • imprint: Frontiers Media SA, 2023
  • Published in: Frontiers in Endocrinology
  • Language: Not determined
  • DOI: 10.3389/fendo.2023.1279828
  • ISSN: 1664-2392
  • Origination:
  • Footnote:
  • Description: <jats:sec><jats:title>Purpose</jats:title><jats:p>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).</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>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 <jats:italic>SDHB</jats:italic> 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.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>The recurrence of PPGLs occurred more frequently in patients with <jats:italic>SDHB</jats:italic> 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.</jats:p></jats:sec>
  • Access State: Open Access