title: Outcome of adrenocortical tumors in children.
abstract: OBJECTIVE
  This study reviews adrenocortical tumors in children to determine factors that significantly affect outcome.
  
  
  METHODS
  An institutional review board-approved retrospective review from 1976 to 2005 identified 23 patients younger than 19 years old with histologic confirmation of adrenocortical carcinoma (ACC) and adenomas.
  
  
  RESULTS
  The mean age of the 23 children was 9.0 +/- 1.6 years; girls predominated (female-to-male ratio = 1.9:1) as did cancers (ACC 16, adenoma 7); tumor hormone production (74%); and advanced stage for disease (66%). All malignancies were more than 2.5 cm. Adrenalectomy, including en bloc resection of adjacent structures (35%) achieved grossly negative margins in 70% of patients. Three patients received chemotherapy or chemoradiation as primary treatment without surgery. There was no perioperative mortality; morbidity was 10% (pneumothorax, acute renal failure, chylous ascites, and thrombocytosis). Surgical cure without adjuvant therapy was achieved for all adenomas and ACC stages I and II. For ACC stage III and IV, median survival was 21 months, 5-year survival was 0%. All advanced-staged ACC received adjuvant therapy. Surgically negative margins conferred a survival advantage.
  
  
  CONCLUSIONS
  Children, especially females with ACC present with large advanced-staged tumors. Surgically negative margins with or without en bloc resection improves survival. The high percentage of children with functioning tumors suggests earlier detection is possible.
authors.affiliation: Division of General and Gastroenterologic Surgery, Department of Surgery, Mayo Clinic Rochester, MN, USA.
authors.forename: Angela M
authors.initials: AM
authors.lastname: Hanna
authors.forename: Tuan H
authors.initials: TH
authors.lastname: Pham
authors.forename: Johanna R
authors.initials: JR
authors.lastname: Askegard-Giesmann
authors.forename: Jayleen M
authors.initials: JM
authors.lastname: Grams
authors.forename: Corey W
authors.initials: CW
authors.lastname: Iqbal
authors.forename: Penny
authors.initials: P
authors.lastname: Stavlo
authors.forename: Christopher R
authors.initials: CR
authors.lastname: Moir
mesh_terms: D000236:Adenoma / Q000401:mortality* / Q000473:pathology / Q000601:surgery* / Q000628:therapy; D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000401:mortality* / Q000473:pathology / Q000601:surgery* / Q000628:therapy; D000315:Adrenalectomy; D018268:Adrenocortical Carcinoma / Q000401:mortality* / Q000473:pathology / Q000601:surgery* / Q000628:therapy; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009367:Neoplasm Staging; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome