Abstract Context Pheochromocytomas and paragangliomas (PPGL) are rare endocrine tumors with high heritability. Carriers of pathogenic variants (PVs) in susceptibility genes face a lifelong risk of recurrence or metastatic disease. Quality of life (QoL) in patients with PPGL, a history of PPGL and PV carriers, remains insufficiently studied. Methods We assessed patient-reported QoL in patients with PPGL before and after surgery and in carriers of susceptibility PVs for the development of PPGLs within the prospective ProsPheo study. QoL was evaluated using standardized questionnaires (SF-12, PHQ-D, GAD-7). Results A total of 202 participants were analyzed. SF-12 physical component summary scores (PCS) differed significantly across subgroups (p=0.006), with the lowest PCS scores in patients with metastatic PPGL (40.3) and unresected head and neck paragnaliomas (HNPGL) (40.0), compared with PV carriers after curative PPGL resection (50.2). Within metastatic disease, hormonally-active tumors showed lower PCS scores than inactive tumors (36.9 vs. 46.2; p=0.03). Perceived physical health in carriers of a PV without history of a PPGL was significantly lower compared to the age-matched general population. Mental health was not significantly impaired across all subgroups. Clinically relevant anxiety was reported in 15.2% of PV carriers and 21.7% of patients with unresected HNPGLs, compared with about 5.9% in the general population. Conclusions Patient-reported physical health was reduced in patients with metastatic PPGLs, unresected HNPGLs, and PV carriers without prior PPGL, whereas patients after curative PPGL resection reported QoL comparable to the general population, regardless of PV status, suggesting that annual follow-up has minimal impact on QoL.
Fischer et al. (2026) studied this question.