American Urological Association - Adrenocortical Carcinoma
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Adrenocortical Carcinoma

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  • Bimodal distribution, first two decades and larger peak at 50s.
  • Most (up to 75%) are unassociated with hormone production and a subset may have Cushing syndrome or evidence of sex hormone overproduction.
  • Gross: Weight usually >100 g; cut surface is variegated with hemorrhage and necrosis (image A).
  • Histology:
    • Diagnosis of malignancy is definitively established when metastatic disease is discovered; however, there are some features that favor a malignancy process such as:
    • High nuclear grade (image B).
    • Venous invasion.
    • Necrosis.
    • Mitosis (>5/50).
    • Diffuse architecture.
    • Capsular invasion.
    • Atypical mitoses.
    • Sinusoidal invasion.
    • Clear cells <25%.
    • Suggested that presence of at least three of these pathologic variables is supportive of malignancy (Weiss scoring system).
  • Immunohistochemistry:
    • Inhibin+, calretinin+ and melan-A+.
    • Synaptophysin-, chromogranin- and S100- (+ in pheochromocytoma)
  • 5-year survival for localized (I and II) (62%) versus advances disease (7%).