International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents

Abstract

Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumours that arise not only in adulthood but also in childhood and adolescence. Up to 70–80% of childhood PPGL are hereditary, accounting for a higher incidence of metastatic and/or multifocal PPGL in paediatric patients than in adult patients. Key differences in the tumour biology and management, together with rare disease incidence and therapeutic challenges in paediatric compared with adult patients, mandate close expert cross-disciplinary teamwork. Teams should ideally include adult and paediatric endocrinologists, oncologists, cardiologists, surgeons, geneticists, pathologists, radiologists, clinical psychologists and nuclear medicine physicians. Provision of an international Consensus Statement should improve care and outcomes for children and adolescents with these tumours. Phaeochromocytomas and paragangliomas (PPGL) are rare endocrine tumours that can affect paediatric patients as well as adults. In this first international Consensus Statement on PPGL in paediatric patients, the authors discuss the diagnosis, management and long-term surveillance of these tumours in children and adolescents.