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Multicenter Study
. 2023 Dec 7:14:1279828.
doi: 10.3389/fendo.2023.1279828. eCollection 2023.

Local recurrence and metastatic disease in pheochromocytomas and sympathetic paragangliomas

Marta Araujo-Castro  1   2 , Iñigo García Sanz  3 , César Mínguez Ojeda  4 , Felicia Hanzu  5 , Mireia Mora  5 , Almudena Vicente  6 , Concepción Blanco Carrera  7 , Paz de Miguel Novoa  8 , María Del Carmen López García  9 , Cristina Lamas  9 , Laura Manjón-Miguélez  10 , María Del Castillo Tous  11 , Pablo Rodríguez de Vera  11 , Rebeca Barahona San Millán  12 , Mónica Recasens  12 , Mariana Tomé Fernández-Ladreda  13 , Nuria Valdés  14 , Paola Gracia Gimeno  15 , Cristina Robles Lazaro  16 , Theodora Michalopoulou  17 , Cristina Álvarez Escolá  18 , Rogelio García Centeno  19 , Verónica Barca-Tierno  20 , Aura D Herrera-Martínez  21 , María Calatayud  22
Affiliations
Multicenter Study

Local recurrence and metastatic disease in pheochromocytomas and sympathetic paragangliomas

Marta Araujo-Castro et al. Front Endocrinol (Lausanne). .

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.

Keywords: SDHB gene; adrenal tumor; catecholamines; metastatic PPGL; recurrent disease.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Study population.
Figure 2
Figure 2
Failure curve for recurrent disease in PPGL.
Figure 3
Figure 3
Survival curves of patients who died and had recurrent disease vs. patients who died and did not have recurrence.
Figure 4
Figure 4
Differences in time free of recurrence between patients with and without pathogenic variants in SDHB.
See this image and copyright information in PMC

References

    1. Garcia-Carbonero R, Matute Teresa F, Mercader-Cidoncha E, Mitjavila-Casanovas M, Robledo M, Tena I, et al. . Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas. Clin Transl Oncol (2021) 23:1995–2019. doi: 10.1007/s12094-021-02622-9 - DOI - PMC - PubMed
    1. Kantorovich V, Pacak K. New insights on the pathogenesis of paraganglioma and pheochromocytoma. F1000Research (2018) 7. doi: 10.12688/F1000RESEARCH.14568.1/DOI - DOI - PMC - PubMed
    1. Araujo-Castro M, Pascual-Corrales E, Nattero Chavez L, Martínez Lorca A, Alonso-Gordoa T, Molina-Cerrillo J, et al. . Protocol for presurgical and anesthetic management of pheochromocytomas and sympathetic paragangliomas: a multidisciplinary approach. J Endocrinol Invest (2021) 44:2545–55. doi: 10.1007/s40618-021-01649-7 - DOI - PubMed
    1. Alrezk R, Suarez A, Tena I, Pacak K. Update of pheochromocytoma syndromes: genetics, biochemical evaluation, and imaging. Front Endocrinol (Lausanne) (2018) 9:515. doi: 10.3389/fendo.2018.00515 - DOI - PMC - PubMed
    1. Parasiliti-Caprino M, Lucatello B, Lopez C, Burrello J, Maletta F, Mistrangelo M, et al. . Predictors of recurrence of pheochromocytoma and paraganglioma: a multicenter study in Piedmont, Italy. Hypertens Res (2020) 43:500–10. doi: 10.1038/s41440-019-0339-y - DOI - PubMed

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