Very rare malignancy of the adrenal cortex

Aetiology

• Rarer than adenomas
• Mainly occurs in adults
• In younger patients can be associated with Li-Fraumeni syndrome
• Equal sex incidence

Pathophysiology

• More likely to be functional - virilising tumours usually malignant
• Can closely resemble adenoma

Histological features

• Large size (>50g, often >20cm)
• Haemorrhage and necrosis
• Frequent mitosis, atypical mitoses
• Lack of clear cells
• Capsular or vascular invasion

Spread of carcinoma

• Local invasion - retroperitoneum, kidney
• Metastasis - usually haemogenous (liver, lung and bone)
• Peritoneum and pleural involvement