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Pediatric pheochromocytoma: current status of diagnostic imaging and treatment procedures

Leslie Peard1, Nicholas G Cost2, Amanda F Saltzman1

  • 1Department of Urology, University of Kentucky, Lexington, Kentucky.

Current Opinion in Urology
|June 28, 2019
PubMed

Insights

Pediatric pheochromocytoma (PCC) often has hereditary causes and aggressive features, differing from adult cases. Genetic testing is crucial for diagnosis, screening, and managing this rare tumor in young patients.

Area of Science:

  • Pediatric Endocrinology
  • Oncology
  • Genetics

Background:

  • Pediatric pheochromocytoma (PCC) is a rare neuroendocrine tumor.
  • Existing knowledge often extrapolates from adult PCC, but pediatric cases present unique characteristics.

Purpose of the Study:

  • To review current data on pediatric pheochromocytoma.
  • To provide updated management recommendations for pediatric PCC.

Main Methods:

  • Literature review of case reports and series on pediatric PCC.
  • Analysis of recent findings on genetic syndromes and metastatic disease treatments.

Main Results:

  • Pediatric PCC frequently involves hereditary predispositions and aggressive or malignant disease.
  • Genetic syndromes are increasingly identified, guiding screening and counseling.
  • Advances in treating metastatic PCC are emerging.
  • Plasma metanephrines and cross-sectional imaging are key for diagnosis.
  • Preoperative medical optimization and surgical resection remain primary treatment modalities.

Conclusions:

  • Genetic testing is pivotal in managing pediatric, adolescent, and young adult PCC.
  • Understanding hereditary factors and aggressive disease is essential for effective pediatric PCC management.
Abstract

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