All-Cause Mortality and Secondary Malignancy in Patients with Pheochromocytoma/Paraganglioma

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that can be fatal if not diagnosed early or adequately managed. This study investigated the risk of all-cause mortality and secondary malignancies in patients with PPGL compared to age- and sex-matched controls without PPGL.

This nationwide population-based cohort study included 1,672 patients with PPGL and 16,790 controls with a median follow-up of approximately 7.1 years. A Cox proportional hazard model was used to estimate the adjusted hazard ratio (HR) and 95% confidence intervals (CI).

The mean age was 46.5 years and 50.5% were male. All-cause mortality was identified in 118 patients with PPGL (7.06%) and 749 controls (4.46%) during the follow-up period. The incidence of all-cause mortality in patients with PPGL was significantly higher than that in controls (9.92 per 1,000 person-years vs. 6.49 per 1,000 person-years, P=0.0003). The risk of all-cause mortality was significantly higher in patients with PPGL than in the controls (HR, 1.372; 95% CI, 1.124 to 1.675). The risk of secondary malignancies was significantly higher in patients with PPGL than in controls (HR, 1.612; 95% CI, 1.33 to 1.954) after adjusting. Among the various cancer subtypes, thyroid and prostate cancers were observed more frequently in PPGL patients, and these findings should be interpreted as descriptive trends.

Patients with PPGL exhibited significantly increased risks of all-cause mortality and secondary malignancies compared with controls, highlighting the importance of appropriate assessment and follow-up.