Determinants of disease-specific survival in patients with and without metastatic pheochromocytoma and paraganglioma

Pheochromocytomas and paragangliomas (PPGLs) have a heterogeneous prognosis, the basis of which remains unclear. We, therefore, assessed disease-specific survival (DSS) and potential predictors of progressive disease in patients with PPGLs and head/neck paragangliomas (HNPGLs) according to the presence or absence of metastases. This retrospective study included 582 patients with PPGLs and 57 with HNPGLs. DSS was assessed according to age, location and size of tumours, recurrent/metastatic disease, genetics, plasma metanephrines and methoxytyramine. Among all patients with PPGLs, multivariable analysis indicated that apart from older age (HR = 5.4, CI = 2.93–10.29, P