Adrenocortical carcinoma

Adrenocortical carcinoma (ACC) is extremely rare – only about 1–2 cases per million people each year. In Germany, that's around 100 people a year.

ACC is a malignant tumour of the adrenal cortex – two small hormone glands above the kidneys that produce important hormones such as cortisol, aldosterone and androgens. Only a few clinics specialise in its treatment.

Around 60 % of tumours are hormone-active. They produce too many hormones, causing symptoms such as weight gain, high blood pressure, skin changes or hair growth.

Because symptoms are often non-specific, ACC is frequently discovered only at an advanced stage – when the tumour is already large or has spread.

The main treatment is surgery to completely remove the affected adrenal gland. The drug mitotane or chemotherapy is often used in addition. So far, there are few promising therapies.

The chances of recovery depend heavily on the stage. Relapses are common, often within the first two years after surgery.

• Stage I–II (localised): five-year survival around 50–60 %
• Stage III (locally advanced): around 35–40 %
• Stage IV (metastatic): usually below 15 %

Because the disease is so rare, there are few large studies. Raising awareness matters, so that patients get the right diagnosis and treatment sooner. The endocrinology department at University Hospital Würzburg is one of Europe's leading centres for this disease.