Predictors of outcome in phaeochromocytomas and paragangliomas

Abstract

Phaeochromocytomas and paragangliomas (PPGLs) are catecholamine-secreting neuroendocrine tumours characterised by high rates of heritability and genetic heterogeneity. Despite advances in the genetic diagnosis and improved understanding of the molecular aberrations underlying these tumours, predictive markers of malignancy remain scarce, limiting the outlook of patients with metastatic PPGL. The identification of robust predictive markers remains the most pressing challenge in PPGL management, so that the potential of targeted therapy to impact patient care can be fully realised.