Journal Article

Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma

Fliedner, Stephanie, Eisenhofer, Graeme, Li, Minghao, Kerstens, Michiel N, Bechmann, Nicole, Timmers, Henri J L M, Pacak, Karel, Berends, Annika M A, Talvacchio, Sara, Robledo, Mercedes, Meuter, Leah, Pamporaki, Christina, Prejbisz, Aleksander, Remde, Hanna, Prodanov, Tamara, Nölting, Svenja, Lenders, Jacques W M

2023

Overview

ContextLong-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. ObjectiveTo investigate the prevalence of recurrence among patients with sporadic compared with hereditary PPGL and to identify predictors of recurrence for sporadic disease. MethodsThis 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. ResultsPrevalence 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. ConclusionPatients 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.

• Publisher: Oxford University Press
• Subject: Adrenal Gland Neoplasms - diagnosis / Adrenal Gland Neoplasms - epidemiology / Adrenal Gland Neoplasms - genetics / Care and treatment / Catecholamines / Clinical / Comparative analysis / Diagnosis / Diseases / Dopamine receptors / Genetic aspects / Genetic screening / Genotype & phenotype / Humans / Kinases / Medical research / Medicine, Experimental / Metastases / Metastasis / Neoplasm Recurrence, Local - epidemiology / Paraganglioma / Paraganglioma - diagnosis / Paraganglioma - epidemiology / Paraganglioma - genetics / Phenotypes / Pheochromocytoma / Pheochromocytoma - diagnosis / Pheochromocytoma - epidemiology / Pheochromocytoma - genetics / Relapse / Retrospective Studies / Tumors