Adrenocortical carcinoma: an orphan disease with many faces

This thesis focuses on research of cancer of the outer layer, the cortex, of the adrenal gland: the adrenal cortex carcinoma. Adrenal cortex carcinoma is a rare form of cancer with poor prognosis. Up to 30% of patients with adrenal cortex cancer present with incurable metastatic disease, stage IV cancer. The only option to cure non-metastatic adrenal cortex carcinoma is to completely cut out the cancer surgically. However, even after successful surgery there is a very high chance that the disease will return. Drug options are limited and with low effectiveness. The drug mitotane plays a leading role in treatment, however this drug has serious side effects and patients perceive the therapy as debilitating. There is therefore an urgent need for additional treatments to improve survival. In order to achieve this, understanding the onset of the disease is particularly important, but also how the disease develops over time. In addition, the current therapy with mitotane should be optimised.