Long-Term Persistence of Glycemic Dysregulation in Patients With a History of Pheochromocytoma/Paraganglioma.

Abstract

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. To determine whether markers of elevated cardiometabolic risk persist in patients following PPGL resection. In this retrospective analysis of a multicenter cohort of patients with PPGLs participating in the prospective ProsPheo study and the ENS@T registry, cardiometabolic risk factors, including glycemic status, dyslipidemia, and body mass index (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 nonfunctioning adrenal adenomas from the ENS@T registry. 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 = .013) compared with 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 < .001), as well as the mean HbA1c (5.63%, SD 0.43%), were significantly higher, compared to a control group with nonfunctioning adrenal adenomas (n = 76) of similar age and BMI (HbA1c 5.45%, SD 0.40%; P = .004). Glycemic disturbances persist long-term after the resection of PPGL.