Incidence

Adrenal gland cancers – otherwise known as adrenocortical carcinomas – are rare (1 in 2,000,000 of all cancers). They can occur at any age, including childhood. The median age of diagnosis is 44 years.

Predisposing Factors

The cause is unknown.

Macroscopic Features

Adrenocortical carcinomas are usually large masses (many exceed 20 cm in diameter) that sit above the kidney and often compress it. They are yellow, often bleeding and cystic, with dead areas within them.

Natural History

Adrenocortical carcinomas tend to invade the adrenal vein, vena cava and lymphatics. Metastases to regional and periaortic lymph nodes are common, as well as haematogenous spread to lungs and other viscera. Bone metastases are uncommon.

Clinical History

60% of patients have symptoms as a result of hormone excess, which may include high blood pressure, diabetes and osteoporosis. Tumours that secrete hormones are called functioning tumours. If the tumour secretes sex hormones, women may experience facial hair or deepening of the voice, while men may experience swelling of the breast and genitals.

Other symptoms include pain in the abdomenweight loss, and weakness.

Clinical Examination

full physical examination is conducted with attention to symptoms caused by hormone excess. An examination of the abdomen will look for masses.

General Investigation

Investigations include appropriate endocrine studies, and a CT and/or MRI of the abdomen. Further specific investigations may include selective angiography and adrenal venography (useful for smaller tumours and distinguishing adrenal from kidney tumours). A PET scan may be used to stage and detect metastases.

Specific Investigations

Specific investigations may include a CT or MRI of the abdomen. Various other investigations (eg. A PET scan) will assess whether the tumour has spread.

The CT scan below indicates a large right adrenal mass outlined in yellow under the normal sized liver (in red). The blue outlining is that of a normal adrenal.

Prognosis

Prognosis depends upon the size of the tumour and its resectability. Metastases occur early and only 30% of diagnosed tumours are confined to the adrenal gland. Patients with confined disease have a better prognosis. The overall 5-year survival for resectable adrenocortical tumours is 40%. The average length of survival for patients with stage IV tumours is usually less than 9 months.