Adrenal cortical carcinoma

Abstract

A review of 28 cases of adrenal cortical carcinoma provides some insights into the natural history of this disease and suggests modifications in current techniques of diagnosis and management. Our patients ranged in age from 1 to 69 years but it is notable that all of our male patients were more than 39 years old. In addition the men tended to have high stage tumors that were nonfunctional. Female patients, on the other hand, were more evenly distributed in terms of stage, function and age at diagnosis. Staging was extremely important and is discussed in some detail. A dismayingly large number of patients were found to have stage 3 or 4 tumor at initial diagnosis, suggesting that aggressive and rapid evaluation and a high index of suspicion are important to optimize survival. Function is helpful but non-function is common and should not dissuade one from the diagnosis. Excretory urography with tomography followed by angiography frequently will provide the diagnosis. Endocrinologic evaluation should be done with alacrity. Survival with high stages of disease is poor and lengthy evaluation should not be allowed to delay prompt and, hopefully, curative surgery. Surgical resection is the key to cure; chemotherapy and radiotherapy provided relatively poor results in our hands although mitotane has shown a 34 to 54 per cent response rate.