The following is a summary of “Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma,” published in the February 2023 issue of Endocrinology & Metabolism by Li, et al.

For a multicenter study, researchers sought to investigate the prevalence of recurrence among patients with sporadic and hereditary pheochromocytoma or paraganglioma (PPGL) and to identify predictors of recurrence for sporadic disease. 

The study involved retrospective data from 1,127 patients with PPGL, including information on sex, age at primary tumor diagnosis, 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 those with negative genetic test results.

The prevalence of recurrence among patients with sporadic PPGL was 14.7%, which 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 the first diagnosis. Multivariable regression analysis revealed 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.

Long-term follow-up was necessary for patients with sporadic PPGL, as evidenced by the 14.7% prevalence of recurrent disease, which included recurrences more than 10 years after the first diagnosis. The type of follow-up could vary depending on the tumor’s size, location, and biochemical profile.

Reference: academic.oup.com/jcem/article-abstract/108/2/397/6747526?redirectedFrom=fulltext