title: Adrenal cortical tumors in children: factors associated with poor outcome.
abstract: OBJECTIVE
  The purpose of this study was to evaluate recurrence and survival outcomes in pediatric adrenal cortical neoplasms.
  
  
  METHODS
  A 90-year retrospective review of children with adrenal cortical neoplasms was performed using multivariate Cox regression analysis to identify factors associated with recurrence and tumor-related mortality.
  
  
  RESULTS
  The evaluable cohort included 29 patients. Twenty-seven underwent resection. Twenty-two (81%) had localized disease, and 5 (19%) had locally advanced disease (all received chemotherapy and 2 of 5 were cured). Two patients presenting with metastatic disease died despite treatment. There were 4 recurrences; all patients died. Tumor-related mortality was 24% (7/29). Kaplan-Meier freedom from recurrence was 85% at 1 year (95% confidence interval, 75%-95%). Multivariate Cox regression revealed that older age (P = .01), higher mitotic rate (P = .005), and necrosis (P < .001) were independent predictors of tumor-related death. Higher mitotic rate (P = .007) and larger tumor size (P = .03) were significant predictors of tumor recurrence.
  
  
  CONCLUSIONS
  Risk factors for poor outcomes in patients with adrenocortical tumors include older age, higher mitotic rate, higher percent necrosis, and larger tumor size. Therefore, the presence of these factors may warrant consideration of adjuvant chemotherapy, even in the absence of advanced disease.
authors.affiliation: Department of Surgery, Children's Hospital Boston, Harvard Medical School, Boston, MA 02115, USA.
authors.forename: Justin D
authors.initials: JD
authors.lastname: Klein
authors.forename: Christopher G
authors.initials: CG
authors.lastname: Turner
authors.forename: Fabienne L
authors.initials: FL
authors.lastname: Gray
authors.forename: David C
authors.initials: DC
authors.lastname: Yu
authors.forename: Harry P
authors.initials: HP
authors.lastname: Kozakewich
authors.forename: Antonio R
authors.initials: AR
authors.lastname: Perez-Atayde
authors.forename: Stephan D
authors.initials: SD
authors.lastname: Voss
authors.forename: David
authors.initials: D
authors.lastname: Zurakowski
authors.forename: Robert C
authors.initials: RC
authors.lastname: Shamberger
authors.forename: Christopher B
authors.initials: CB
authors.lastname: Weldon
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D002423:Cause of Death; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D015999:Multivariate Analysis; D009361:Neoplasm Invasiveness / Q000473:pathology; D009364:Neoplasm Recurrence, Local / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D009367:Neoplasm Staging; D019635:Neurosurgical Procedures / Q000379:methods; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis