Paediatric phaeochromocytoma and paraganglioma: A clinical update

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...

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Published in:Clinical endocrinology (Oxford) 2024-11, Vol.101 (5), p.446-454
Main Authors: Nazari, Matthew A., Jha, Abhishek, Kuo, Mickey J. M., Patel, Mayank, Prodanov, Tamara, Rosenblum, Jared S., Talvacchio, Sara, Derkyi, Alberta, Charles, Kailah, Pacak, Karel
Format: Article
Language:English
Subjects:
Adrenal Gland Neoplasms - diagnosis
Adrenal Gland Neoplasms - therapy
Child
disease management
genetics
Humans
molecular imaging
paediatric
Paraganglioma
Paraganglioma - diagnosis
Paraganglioma - therapy
pathology
Patients
Pediatrics
phaeochromocytoma
Pheochromocytoma
Pheochromocytoma - diagnosis
Pheochromocytoma - therapy
Prognosis
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Summary: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.
ISSN:0300-0664
1365-2265
1365-2265
DOI:10.1111/cen.14955