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Updated: Jul 20, 2025

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Paediatric phaeochromocytoma and paraganglioma: A clinical update

Matthew A Nazari1, Abhishek Jha1, Mickey J M Kuo1,2

  • 1Developmental Endocrinology, Metabolism, Genetics and Endocrine Oncology Affinity Group, Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA.

Clinical Endocrinology
|July 29, 2023
PubMed

Insights

Paediatric phaeochromocytomas and paragangliomas (PPGLs) are rare but serious. Early diagnosis and prompt treatment of these pediatric tumors lead to favorable outcomes.

Area of Science:

  • Pediatric Oncology
  • Endocrinology
  • Rare Tumors

Background:

  • Paediatric phaeochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors.
  • These tumors can cause significant morbidity and mortality in children.
  • Early recognition of clinical signs is crucial for timely diagnosis.

Purpose of the Study:

  • To review state-of-the-art management concepts for pediatric PPGLs.
  • To highlight key features through four exemplary case studies.
  • To emphasize the importance of prompt intervention in pediatric PPGL cases.

Main Methods:

  • Review of four exemplary pediatric cases of PPGLs.
  • Synthesis of current management strategies and concepts.
  • Focus on clinical presentation and diagnostic considerations.

Main Results:

  • Pediatric PPGLs can present overtly, aiding early diagnosis.
  • Timely and effective intervention is associated with favorable prognosis.
  • Case examples illustrate key aspects of diagnosis and management.

Conclusions:

  • Prompt diagnosis and effective management are critical for improving outcomes in pediatric PPGLs.
  • Understanding the clinical presentation aids in early detection.
  • Current management strategies offer a favorable prognosis when implemented timely.

Keywords:
disease managementgeneticsmolecular imagingpaediatricparagangliomapathologyphaeochromocytoma

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