Supplemental Appendix for manuscript 'Recurrent disease in patients with sporadic pheochromocytoma and paraganglioma

Bechmann, Nicole

Berends, Annika M.A.

Eisenhofer, Graeme

Fliedner, Stephanie

Kerstens, Michiel N.

Lenders, Jacques W.M.

Li, Minghao

Meuter, Leah

Nölting, Svenja

Pacak, Karel

Pamporaki, Christina

Prejbisz, Aleksander

Prodanov, Tamara

Remde, Hanna

Robledo, Mercedes

Talvacchio, Sara

Timmers, Henri J.L.M.

English

Context: Long-term follow-up has been recommended for patients with pheochromocytoma or paraganglioma (PPGL) due to potential for recurrent disease. However, the need to follow patients with sporadic PPGL has recently become controversial.Objective: To investigate the prevalence of recurrence among patients with sporadic compared with hereditary PPGL and to identify predictors of recurrence for sporadic disease.Methods: This multicenter study included retrospective data from 1127 patients with PPGL. In addition to sex and age at primary tumor diagnosis, clinical information included location, size, and catecholamine phenotype of primary tumors, genetic test results, and subsequent development of recurrent and/or metastatic disease. Patients with sporadic PPGL were defined as those with negative genetic test results.Results: Prevalence of recurrence among patients with sporadic PPGL (14.7%) was lower (P &lt; 0.001) than for patients with pathogenic variants that activate pseudohypoxia pathways (47.5%), but similar to those with variants that activate kinase pathways (14.9%). Among patients with sporadic recurrent PPGL, 29.1% and 17.7% were respectively diagnosed at least 10 and 15 years after first diagnosis. Multivariable regression analysis showed that a noradrenergic/dopaminergic phenotype (HR 2.73; 95% CI, 1.553-4.802; P &lt; 0.001), larger size (HR 1.82; 95% CI, 1.113-2.962; P = 0.017) and extra-adrenal location (HR 1.79; 95% CI, 1.002-3.187; P = 0.049) of primary tumors were independent predictors of recurrence in sporadic PPGL.Conclusion: Patients with sporadic PPGL require long-term follow-up, as supported by the 14.7% prevalence of recurrent disease, including recurrences at more than 10 years after first diagnosis. The nature of follow-up could be individualized according to tumor size, location, and biochemical phenotype.</p

Proceedings - University of Groningen

Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma

ARTS repository - University of Groningen

Kerstens, Michiel

Timmers, Henri

Berends, Annika

Lenders, Jacques

NEUROSURGERY ENTHUSIASTIC WOMEN SOCIETY

Recurrent disease in patients with sporadic pheochromocytoma and paraganglioma

Dissertations of the University of Groningen

https://pure.rug.nl/ws/files/764389979/dgac563.pdf

Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma

Abstract

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Proceedings - University of Groningen

ARTS repository - University of Groningen

Proceedings - University of Groningen

NEUROSURGERY ENTHUSIASTIC WOMEN SOCIETY

NEUROSURGERY ENTHUSIASTIC WOMEN SOCIETY

NEUROSURGERY ENTHUSIASTIC WOMEN SOCIETY

Dissertations of the University of Groningen

Dissertations of the University of Groningen

NEUROSURGERY ENTHUSIASTIC WOMEN SOCIETY

ARTS repository - University of Groningen