A case of recurrent adrenocortical carcinoma, with observations on long-term o,p'-DDD therapy and complications

This report describes a patient with a recurring, one stemline-aneuploid, adrenocortical carcinoma. The condition showed a number of unusual characteristics over a period of 22 yr. It changed from a biochemically functioning, low-grade malignant tumour into a non-functioning malignancy with pronounced mitotic activity, accompanied by an ovarian carcinosarcoma 1 yr before death. Quality of life was reasonable for many years despite chemotherapy, consisting of a total of almost 10 kg of o,p'-DDD administered over a period of 8 yr, and the subsequent side effects (e.g. low T4; increased bleeding time). A reduced mineralocorticoid activity, induced by o,p'-DDD, was reversed after discontinuation of o,p'-DDD treatment. During o,p'-DDD administration the substitution requirements for both hydrocortisone and fludrocortisone acetate increased, leading to periods of hypoadrenocorticism with prerenal uraemia.