title: Partial adrenalectomy minimizes the need for long-term hormone replacement in pediatric patients with pheochromocytoma and von Hippel-Lindau syndrome.
abstract: OBJECTIVE
  Children with von Hippel-Lindau syndrome (VHL) are at an increased risk for developing bilateral pheochromocytomas. In an effort to illustrate the advantage of partial adrenalectomy (PA) over total adrenalectomy in children with VHL, we report the largest single series on PA for pediatric patients with VHL, demonstrating a balance between tumor removal and preservation of adrenocortical function.
  
  
  METHODS
  From 1994 to 2011, a prospectively maintained database was reviewed to evaluate 10 pediatric patients with hereditary pheochromocytoma for PA. Surgery was performed if there was clinical evidence of pheochromocytoma and if normal adrenocortical tissue was evident on preoperative imaging and/or intraoperative ultrasonography. Perioperative data were collected, and patients were observed for postoperative steroid use and tumor recurrence.
  
  
  RESULTS
  Ten pediatric patients with a diagnosis of VHL underwent 18 successful partial adrenalectomies (4 open, 14 laparoscopic). The median tumor size removed was 2.6 cm (range, 1.2-6.5 cm). Over a median follow-up of 7.2 years (range, 2.6-15.8 years), additional tumors in the ipsilateral adrenal gland were found in 2 patients. One patient underwent completion adrenalectomy, and 1 underwent a salvage PA with resection of the ipsilateral lesion. One patient required short-term steroid replacement therapy. At last follow-up, 7 patients had no radiographic or laboratory evidence of pheochromocytoma.
  
  
  CONCLUSIONS
  At our institution, PA is the preferred form of management for pheochromocytoma in the (VHL) pediatric population. This surgical approach allows for removal of tumor while preserving adrenocortical function and minimizing the adverse effects of long-term steroid replacement on puberty and quality of life.
authors.affiliation: Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892-1210, USA.
authors.forename: Dmitry
authors.initials: D
authors.lastname: Volkin
authors.forename: Nitin
authors.initials: N
authors.lastname: Yerram
authors.forename: Faisal
authors.initials: F
authors.lastname: Ahmed
authors.forename: Dawud
authors.initials: D
authors.lastname: Lankford
authors.forename: Angelo
authors.initials: A
authors.lastname: Baccala
authors.forename: Gopal N
authors.initials: GN
authors.lastname: Gupta
authors.forename: Anthony
authors.initials: A
authors.lastname: Hoang
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Nix
authors.forename: Adam R
authors.initials: AR
authors.lastname: Metwalli
authors.forename: David M
authors.initials: DM
authors.lastname: Lang
authors.forename: Gennady
authors.initials: G
authors.lastname: Bratslavsky
authors.forename: W Marston
authors.initials: WM
authors.lastname: Linehan
authors.forename: Peter A
authors.initials: PA
authors.lastname: Pinto
mesh_terms: D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000209:etiology / Q000601:surgery*; D000309:Adrenal Insufficiency / Q000188:drug therapy / Q000209:etiology / Q000517:prevention & control*; D000315:Adrenalectomy / Q000379:methods*; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D020249:Hormone Replacement Therapy; D006801:Humans; D008297:Male; D010673:Pheochromocytoma / Q000209:etiology / Q000601:surgery*; D011183:Postoperative Complications / Q000188:drug therapy / Q000517:prevention & control*; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000601:surgery*
chemical_list: D013256:Steroids