Recent advances in the diagnosis and treatment of pheochromocytoma in children

Am J Surg. 2007 Dec;194(6):792-6; discussion 796-7. doi: 10.1016/j.amjsurg.2007.08.028.


Background: The purpose of this study was to analyze the effects of changes in the diagnosis and treatment of pheochromocytoma in a pediatric population.

Methods: We reviewed the medical records of all children who had resection of pheochromocytoma or paraganglioma at a major children's hospital since 1968.

Results: Fifteen children underwent surgery at 11.9 +/- 4.2 years of age. Presenting symptoms included headache, hypertension, and sweating. Three children had a mutation of the succinate dehydrogenase enzyme, and 1 child had nonsyndromic, familial pheochromocytoma. The most sensitive diagnostic modalities included 24-hour urinary and plasma norepinephrine and 24-hour urinary total metanephrines, magnetic resonance imaging, and 123I-meta-iodobenzylguanidine scintigraphy. Laparoscopic cortical-sparing adrenalectomy was performed in 3 patients with von Hippel-Lindau disease. Compared with those with open procedures (n = 7), patients who had laparoscopic resection (n = 5) had a statistically shorter hospital length of stay, and time to eating ambulation.

Conclusions: The addition of 123I-meta-iodobenzylguanidine scanning, genetic testing, and laparoscopic surgery has changed the diagnosis and treatment of pheochromocytoma in children. Laparoscopic cortical-sparing adrenalectomy can be accomplished safely and is the preferred treatment for children at risk for multifocal disease.

MeSH terms

  • Adolescent
  • Adrenal Gland Neoplasms / diagnosis*
  • Adrenal Gland Neoplasms / surgery*
  • Adrenalectomy
  • Child
  • Disease-Free Survival
  • Female
  • Humans
  • Laparoscopy
  • Length of Stay
  • Male
  • Paraganglioma / diagnosis*
  • Paraganglioma / surgery*
  • Paraganglioma, Extra-Adrenal / diagnosis
  • Paraganglioma, Extra-Adrenal / surgery
  • Pheochromocytoma / diagnosis
  • Pheochromocytoma / surgery
  • Retrospective Studies
  • Sensitivity and Specificity