Pamporaki Christina, Casey Ruth T
Medical Clinic III, University Hospital Carl Gustav Carus and Medical Faculty, TU Dresden, Germany.
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.
Best Pract Res Clin Endocrinol Metab. 2025 Jan;39(1):101957. doi: 10.1016/j.beem.2024.101957. Epub 2024 Nov 14.
Phaeochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumours which arise from chromaffin cells of the adrenal medulla or extra-adrenal autonomic ganglia. PPGL most commonly present in adulthood but can arise in childhood and adolescence with an estimated annual incidence of 0.5 cases per million children per year. There have been significant advances in the diagnosis and management of PPGL over the past 2-3 decades based largely on the study of adult patients. These advances in clinical knowledge can be applied to paediatric patients but like other cancers, paediatric PPGL must be viewed as a distinct subset with their own specific challenges and opportunities for improved clinical care. This review article provides an overview on the diagnosis and management of PPGL in children focusing on recent international guidance.
嗜铬细胞瘤和副神经节瘤（PPGL）是罕见的神经内分泌肿瘤，起源于肾上腺髓质或肾上腺外自主神经节的嗜铬细胞。PPGL最常见于成年期，但也可发生于儿童和青少年期，估计每年每百万儿童中的发病率为0.5例。在过去20至30年中，基于对成年患者的研究，PPGL的诊断和管理取得了重大进展。这些临床知识的进展可应用于儿科患者，但与其他癌症一样，儿科PPGL必须被视为一个独特的亚组，有其自身特定的挑战和改善临床护理的机会。这篇综述文章概述了儿童PPGL的诊断和管理，重点关注近期的国际指南。
