Adrenocortical Carcinoma

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
notion image

Spread of carcinoma

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

Clinical presentation

  • Hormonal effects
  • Abdominal mass effects
  • Carcinomas with necrosis can cause fever

Investigations

Imaging

  • CT
  • MRI

Biopsy

  • Distinction between benign and malignant can be difficult
  • Metastasis is the only definate criterion for malignancy

Management

  • Resection with adjuvant therapy (if not metastatic)