Adrenocortical carcinoma: diagnosis, evaluation and treatment

Abstract

Purpose: We describe the presenting features, imaging methods, prognosis of and treatment approach to adrenocortical carcinoma.

Materials and methods: A comprehensive literature review of adrenal carcinoma was performed using PubMed and a review of current urology, oncology, radiology and pathology journals.

Results: The incidence of adrenocortical tumors has been reported to be approximately 2/1 x 10(6) population, although this value may be an underestimate due to an increase in the incidence of unexpected adrenal masses or incidentalomas in the last decade. There has been a bimodal age incidence of adrenal carcinoma with most patients 40 to 50 years old in the studies reviewed. Overall the prevalence of adrenal tumors was slightly higher in females with most presenting with Cushing's syndrome. Of the 602 adrenal tumors reviewed 62% were functional and 38% were nonfunctional. Modern day imaging, including ultrasound, computerized tomography (CT) and magnetic resonance imaging, have greatly improved the diagnosis and staging of adrenal carcinoma. Surgical removal of a localized tumor and absent metastatic disease were associated with improved survival. Medical therapy with mitotane and its successors in patients with metastatic disease appear to be of little benefit for improving survival.

Conclusions: Based on a review of the literature we recommend that hormone levels should be determined in symptomatic and asymptomatic patients with adrenal masses. CT should be the first study done to define an adrenal mass. All solid incidentalomas greater than 5 cm. on CT or magnetic resonance imaging should be removed surgically. The best chance of survival was achieved by surgical extirpation with the value of adjuvant therapy yet to be determined.