Paediatric phaeochromocytoma and paraganglioma: A clinical update

作者：Matthew A. Nazari, Abhishek Jha, Mickey J. M. Kuo, Mayank Patel, Tamara Prodanov, Jared S. Rosenblum, Sara Talvacchio, Alberta Derkyi, Kailah Charles, Karel Pacák · 发表于：Clinical Endocrinology · 年份：2023 · DOI：10.1111/cen.14955 · 被引用次数：8 · 研究领域：Adrenal and Paraganglionic Tumors、Neuroblastoma Research and Treatments、Pituitary Gland Disorders and Treatments

Paediatric phaeochromocytomas and paragangliomas (PPGLs), though rare tumours, are associated with significant disability and death in the most vulnerable of patients early in their lives. However, unlike cryptogenic and insidious disease states, the clinical presentation of paediatric patients with PPGLs can be rather overt, allowing early diagnosis, granted that salient findings are recognized. Additionally, with prompt and effective intervention, prognosis is favourable if timely intervention is implemented. For this reason, this review focuses on four exemplary paediatric cases, succinctly emphasizing the now state-of-the-art concepts in paediatric PPGL management.