Background: Adrenocortical carcinoma is a highly malignant tumour with a poor prognosis. Surgery is the treatment of choice, with repeated procedures if necessary.
Material and methods: A 39-year-old woman presented with a pronounced Cushing's syndrome with virilizsation. Investigations showed an adrenocortical carcinoma with a diameter of 12 cm and two large metastases in the liver. She was admitted for palliative surgery. The primary tumour and one liver metastasis were resected. Three months later a right hepatectomy was performed, with excision of the remaining liver metastasis. Mitotane tablets were given all the time.
Results: After the first operation the patient's clinical condition improved radically. Two years later she was clinically healthy without any tumours and normal hormone levels. After 27 months a relapse in the liver was found by computed tomography, and after 33 months steroid hormone precursors again increased. Radiotherapy to the liver metastasis was given, and at 48 months the patient is still healthy and has no relapse.
Interpretation: Patients with adrenocortical carcinoma should be treated with extensive surgery, if necessary with repeated resections. Additional chemotherapy should be considered. This case confirms that surgical therapy has effect on metastasizing and hormone producing adrenal carcinoma and we consider that intensive surgery has improved this patient's prognosis.