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Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma

Authors: Li, Minghao; Prodanov, Tamara; Meuter, Leah; Kerstens, Michiel N; Bechmann, Nicole; Prejbisz, Aleksander; Remde, Hanna; +10 Authors

Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma

Abstract

Abstract 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 < 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 < 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.

Countries
Netherlands, Spain, Switzerland
Keywords

recurrence, Pheochromocytoma, paraganglioma, sporadic, metastases, recurrence, disease free period, 10265 Clinic for Endocrinology and Diabetology, Adrenal Gland Neoplasms, Radboud University Medical Center, 610 Medicine & health, Pheochromocytoma, pheochromocytoma, Paraganglioma, paraganglioma, sporadic, disease free period, Humans, Radboudumc 16: Vascular damage Internal Medicine, Neoplasm Recurrence, Local, metastases, Retrospective Studies

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selected citations
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This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
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popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
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