Department of Medical Genetics, University of Cambridge and NIHR Cambridge Biomedical Research Centre, Cambridge, UK.
Department of Endocrinology, Cambridge Cancer Centre and Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
Nat Rev Endocrinol. 2024 Dec;20(12):729-748. doi: 10.1038/s41574-024-01024-5. Epub 2024 Aug 15.
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.
嗜铬细胞瘤和副神经节瘤（PPGL）是罕见的神经内分泌肿瘤，不仅发生在成年期，也发生在儿童和青少年期。多达 70-80%的儿童 PPGL 是遗传性的，与成年患者相比，儿童患者转移性和/或多灶性 PPGL 的发病率更高。肿瘤生物学和管理方面的关键差异，以及儿科患者罕见疾病发病率和治疗挑战与成年患者相比，都需要密切的专家跨学科团队合作。团队理想情况下应包括成人和儿科内分泌学家、肿瘤学家、心脏病专家、外科医生、遗传学家、病理学家、放射科医生、临床心理学家和核医学医师。提供国际共识声明应改善这些肿瘤患儿的护理和结局。
