American Urological Association - Adrenocortical Carcinoma
- 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).
- 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.
- 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).
- Inhibin+, calretinin+ and melan-A+.
- Synaptophysin-, chromogranin- and S100- (+ in pheochromocytoma)
- 5-year survival for localized (I and II) (62%) versus advances disease (7%).