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Paediatric phaeochromocytoma and paraganglioma: A clinical update.

Matthew A NazariAbhishek JhaMickey J M KuoMayank PatelTamara ProdanovJared S RosenblumSara TalvacchioAlberta DerkyiKailah CharlesKarel Pacak
Published in: Clinical endocrinology (2023)
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.
Keyphrases
  • intensive care unit
  • emergency department
  • randomized controlled trial
  • end stage renal disease
  • chronic kidney disease
  • ejection fraction
  • newly diagnosed
  • prognostic factors
  • patient reported