Adrenocortical carcinomas are very rare malignancies of the cortical adrenal gland. Adrenocortical adenomas are far more common but are usually non-functional.
Epidemiology
These are rare tumours that can occur at any age.
Aetiology and Pathogenesis
Most cases are sporadic but the disease is associated with two germline mutations:
- Li Fraumeni syndrome (germline mutation of one TP53 gene) is associated with an increased risk of this disease, in addition to multiple other malignancies including soft tissue sarcoma and breast cancer
- Beckwith-Weidermann syndrome (rearrangement of 11p15 region) is an overgrowth syndrome that is associated with a number of malignancies in childhood including nephroblastoma and embryonal rhabdomyosarcoma
Natural History
Functional lesions may present with virilisation in women or precocious puberty in boys. In adult men or in non-functional tumours the disease may be clinically silent and late presentations are common. Small, non-functional tumours are very difficult to differentiate from non-functional benign adenomas which represent the majority of incidental adrenal masses on CT.
Tumour/Normal Tissue Features
The tumour is frequently large, necrotic and haemorrhagic macroscopically. Local invasion of vessels is common. Microscopically there is variation from well differentiated (resembling adeonoma with eosinophilic or vacuolated cytoplasm) to markedly anaplastic with massive giant cells.
