Adrenal cortical carcinoma - a case series and literature review of aggressive adrenal incidentalomas.

Objective: Adrenal cortical carcinoma (ACC) is a rare and aggressive endocrine malignancy. Clinical symptoms are mainly related to excess hormone secretion. Hypercortisolism and virilisation are among the most common presentations.

Methods: We report a case series of five patients with ACC, three of which presented as adrenal incidentalomas. Additionally, a literature review on current diagnosis and management of ACC was performed.

Results: ACCs are often incidentally detected because of the liberal use of medical imaging. Management of ACC remains challenging, and the poor prognosis makes early diagnosis of crucial importance to increase chances of a better outcome. Biochemical evaluation should be performed to diagnose hormonally active tumours.

Conclusion: Surgery is the main and only potentially curative treatment option. Adjuvant treatment with mitotane may improve survival and is indicated for patients with a perceived high risk of recurrence. Aggressive cytotoxic therapy should be given to patients with an unfavourable prognosis.