• Rare, 25 cases annually in US
  
• Usually hormone-related symptoms with virilization, Cushing’s syndrome and mixed endocrine syndromes; rarely feminization or Conn’s syndrome
  
• 75% of adrenocortical neoplasms in children are carcinomas, although only 31% of histologically malignant tumors had clinically malignant behavior
  
• Malignant behavior associated with vena cava invasion, tumor necrosis, > 15 mitotic figures/20 HPF, although cannot rely on a single histological feature
  
• Proposed criteria for malignancy in children: > 400g, > 10.5 cm, extension into adjacent soft tissue / organs, extension into vena cava, capsular invasion, vascular invasion, tumor necrosis, > 15 mitotic figures/20 HPF and atypical mitotic figures (Am J Surg Pathol 2003;27:867)
  

• Mean 9 cm, range 2-20 cm
  

• Inhibin, vimentin, CK5, p53 (focal) and Ki-67 (focal)
  

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