title: Adrenal cortical tumours: 25 years' experience at the Royal Children's Hospital, Melbourne.
abstract: OBJECTIVE
  Adrenal cortical tumours remain a rare entity with inconsistent consensus about treatment and follow up. This article reviews 25 years of experience in the Royal Children's Hospital, Melbourne, Australia.
  
  
  METHODS
  All records with the diagnosis of adrenal adenoma or carcinoma between 1976 and 2001 were reviewed, excluding tumours of the adrenal medulla. Details were recorded for age, gender, family history, presentation, biochemistry, imaging, histology, diagnosis, treatment and outcome.
  
  
  RESULTS
  Twelve children (six boys, six girls) were diagnosed with adrenal cortical tumours in this period. Median age at diagnosis was 2.5 years (range 0.5-15.6 years). Six of the 12 children presented with virilization. The remaining six identified by ultrasound performed for hemi-hypertrophy (2), hypertension (2), and fever with abdominal pain (2). Five children had a family history of tumour and two of these five had a p53 mutation demonstrated on molecular genetic analysis. Tumours in five of the 12 children were defined as malignant on histology. Surgery was deemed curative in nine cases. Only one case required further surgery and two required chemotherapy. Time since diagnosis ranged from 1 to 25.8 years. Two children died from complications of the tumour. One other child died following development of a second tumour.
  
  
  CONCLUSIONS
  Adrenal cortical tumours should always be considered in the differential for adrenal hormone excess. New information provided by mutational analysis may predict ongoing risks. Lifelong regular follow up is required.
authors: Department of Endocrinology and Diabetes, Royal Children's Hospital, Melbourne, Victoria, Australia.
authors: C M
authors: CM
authors: McDonnell
authors: M R
authors: MR
authors: Zacharin
mesh_terms: D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000453:epidemiology* / Q000473:pathology* / Q000601:surgery; D000315:Adrenalectomy / Q000379:methods; D018246:Adrenocortical Adenoma / Q000453:epidemiology* / Q000473:pathology* / Q000601:surgery; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D005260:Female; D006776:Hospitals, Pediatric; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000473:pathology* / Q000601:surgery; D009367:Neoplasm Staging; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D018570:Risk Assessment; D017678:Sex Distribution; D016019:Survival Analysis; D013997:Time Factors; D014505:Urban Population; D014739:Victoria / Q000453:epidemiology