Long-term persistence of glycemic dysregulation in patients with a history of pheochromocytoma/paraganglioma

Context: Pheochromocytomas and paragangliomas (PPGLs) are rare endocrine tumors that frequently produce catecholamines. Catecholamine-induced cardiometabolic complications substantially contribute to increased morbidity and mortality in PPGL patients prior to surgical resection.

Objective: To determine whether markers of elevated cardiometabolic risk persist in patients following PPGL resection.

Design: Retrospective analysis of a multicenter cohort of patients with PPGLs participating in the prospective ProsPheo study and the ENS@T registry.

Methods: Cardiometabolic risk factors, including glycemic status, dyslipidemia, and BMI, were assessed in patients with PPGL at diagnosis and during follow-up. Patients with a history of resected PPGL were compared to a control group with non-functioning adrenal adenomas (NFAA) from the ENS@T registry.

Results: Patients with a present PPGL or a history of PPGL (n=188), a metastatic PPGL (n=27) or a known susceptibility gene pathogenic variant (PV) for the development of PPGL without a history of PPGL (n=44), were included. We compared the asymptomatic PV carriers to patients with a history of PPGL: those with a history of PPGL showed a significantly higher prevalence of hyperglycemic disorders (p=0.013) compared to asymptomatic PV carriers. In patients with a history of PPGL and at least 12 months of follow-up post-surgery (n=113), the prevalence of hyperglycemic disorders (p<0.001), as well as the mean HbA1c (5.63%, SD 0.43%), were significantly higher, compared to a control group with NFAA (n=76) of similar age and BMI (HbA1c 5.45%, SD 0.40%; p = 0.004).

Conclusions: Glycemic disturbances persist long-term after the resection of PPGL.