title: Recent advances in the diagnosis and treatment of pheochromocytoma in children.
abstract: 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.
authors.affiliation: Michael E. DeBakey Department of Surgery, Baylor College of Medicine, 6621 Fannin, CCC650.00, Houston, TX 77030, USA.
authors.forename: Andrew D
authors.initials: AD
authors.lastname: Ludwig
authors.forename: Daniel I
authors.initials: DI
authors.lastname: Feig
authors.forename: Mary L
authors.initials: ML
authors.lastname: Brandt
authors.forename: M John
authors.initials: MJ
authors.lastname: Hicks
authors.forename: Megan E
authors.initials: ME
authors.lastname: Fitch
authors.forename: Darrell L
authors.initials: DL
authors.lastname: Cass
mesh_terms: D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000175:diagnosis* / Q000601:surgery*; D000315:Adrenalectomy; D002648:Child; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D010535:Laparoscopy; D007902:Length of Stay; D008297:Male; D010235:Paraganglioma / Q000175:diagnosis* / Q000601:surgery*; D010236:Paraganglioma, Extra-Adrenal / Q000175:diagnosis / Q000601:surgery; D010673:Pheochromocytoma / Q000175:diagnosis / Q000601:surgery; D012189:Retrospective Studies; D012680:Sensitivity and Specificity