title: Clinical and histopathological features of adrenocortical neoplasms in children: retrospective review from a single specialist center.
abstract: OBJECTIVE
  Adrenocortical tumors (ACTs) are rare in children and the extent to which histopathological features can predict clinical behavior remains uncertain. The aim of this study was to investigate the relationship between histopathological features (Weiss score), surgical approach, tumor size, underlying genetic predisposition syndrome, and outcome.
  
  
  METHODS
  Twenty-nine ACTs treated at our institution between 1987 and 2011 were identified from a histopathology database. The histological features were categorized using the Weiss scoring system. For tumor staging, the UKCCSG staging system was utilized.
  
  
  RESULTS
  At a median follow-up of 25 months, 19 patients (65.5%) survived without evidence of disease and 10 patients (35.5%) had died. There was a strong association between high Weiss score and both large tumor size (P<0.01) and adverse outcome (P<0.01). Outcome for stage I and IIA disease was significantly better compared to higher stage disease and/or tumor rupture (P<0.01).
  
  
  CONCLUSIONS
  There is an association between high Weiss score, large tumor size, underlying genetic predisposition syndrome and an adverse outcome for pediatric ACTs. Regardless of histopathological findings, complete surgical resection, without tumor spillage, is optimal for survival. Genetic evaluation is recommended in patients with ACTs, particularly those with a high Weiss score.
authors.affiliation: Department of Paediatric Urology, Great Ormond Street Hospital for Children and Institute of Child Health, London WC1N 3JH, UK.
authors.forename: Akiko
authors.initials: A
authors.lastname: Sakoda
authors.affiliation: Department of Paediatric Urology, Great Ormond Street Hospital for Children and Institute of Child Health, London WC1N 3JH, UK.
authors.forename: Imran
authors.initials: I
authors.lastname: Mushtaq
authors.affiliation: Department of Oncology, Great Ormond Street Hospital for Children and Institute of Child Health, London WC1N 3JH, UK.
authors.forename: Gill
authors.initials: G
authors.lastname: Levitt
authors.affiliation: Department of Histopathology, Great Ormond Street Hospital for Children and Institute of Child Health, London WC1N 3JH, UK. Electronic address: Neil.Sebire@gosh.nhs.uk.
authors.forename: Neil J
authors.initials: NJ
authors.lastname: Sebire
mesh_terms: D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000188:drug therapy / Q000453:epidemiology* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D000315:Adrenalectomy; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006776:Hospitals, Pediatric / Q000706:statistics & numerical data; D006801:Humans; D007431:Intraoperative Complications / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008131:London / Q000453:epidemiology; D008297:Male; D060787:Neoplasm Grading; D009367:Neoplasm Staging; D009386:Neoplastic Syndromes, Hereditary / Q000175:diagnosis / Q000453:epidemiology; D011379:Prognosis; D012189:Retrospective Studies; D012421:Rupture / Q000453:epidemiology; D062606:Tertiary Care Centers / Q000706:statistics & numerical data; D047368:Tumor Burden; D014770:Virilism / Q000209:etiology
chemical_list: D000970:Antineoplastic Agents
keywords: Adrenocortical neoplasm; Genetic predisposition syndrome; Tumor rupture; Weiss score