Paediatric phaeochromocytoma and paraganglioma: A clinical update

Clin Endocrinol (Oxf). 2024 Nov;101(5):446-454. doi: 10.1111/cen.14955. Epub 2023 Jul 28.

Abstract

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.

Keywords: disease management; genetics; molecular imaging; paediatric; paraganglioma; pathology; phaeochromocytoma.

Publication types

  • Review

MeSH terms

  • Adrenal Gland Neoplasms* / diagnosis
  • Adrenal Gland Neoplasms* / therapy
  • Child
  • Humans
  • Paraganglioma* / diagnosis
  • Paraganglioma* / therapy
  • Pheochromocytoma* / diagnosis
  • Pheochromocytoma* / therapy
  • Prognosis