Metastatic adrenocortical carcinoma causing Cushing's syndrome

Abstract

Adrenocortical carcinoma is a very rare malignancy with an estimated incidence of only two cases per million populations per year. The prognosis of patients with advanced disease is unfavourable. A 70 year old previously healthy female presented with symptoms of weight gain mainly of the trunk and face, alopecia and petechia starting two years prior to admission. In addition, arterial hypertension, diabetes and hypercholesterolemia were diagnosed. Urinary free cortisol (635 nmol/d, range <300) and morning cortisol (1472 nmol/l, range 140–600) were elevated. ACTH was suppressed and CRH stimulation test was negative. Dexamethasone suppression test showed no drop in cortisol levels. A tumour mass (4×5cm) localized to the left adrenal gland with multiple pulmonary metastases was identified by CT scan. Histology revealed a well differentiated adrenocortical carcinoma. After surgical removal of the tumour three cycles of adjuvant chemotherapy with etoposide, doxorubicine and cisplatin associated with mitotane were administered. Urine cortisol was used as an tumour marker and decreased after chemotherapy, although computertomography showed a progress of the pulmonary metastases without local tumour recurrence. Therefore chemotherapy was switched to streptozotocine and mitotane. An octreotide scan to assess the possibility of adding a somatostatin receptor analogue was negative. Currently the patient remains healthy and in good clinical condition almost three years after the onset of the first symptoms.