[Diagnosis and therapy of hormonally active cancer of the adrenal cortex. Study of 5 cases].

Adrenocortical carcinomas are very rare tumors. The diagnosis and therapeutic results in 5 female patients are reported. In 4 cases the tumor displayed biochemical and clinical features of Cushing's syndrome, while one was an adrenocortical carcinoma with hypersecretion of aldosterone only. The mean age was 53.6 +/- 8.2 years. In all four cases with Cushing's syndrome the diagnosis was established by measurement of urinary free cortisol extraction rates and excretion rates of 17-ketogenic steroids (mean 1794.5 +/- 1968 micrograms/24 h for free cortisol and 50.5 +/- 24.9 mg/24 h for 17-ketogenic steroids), while the excretion of 17-OH-steroids was in the normal range in one case with hypercortisolism. The patient with mineralocorticoid excess showed markedly elevated plasma aldosterone levels (840 pg/ml) and undetectable plasma renin activity (less than 0.2 ng/ml/3 h), but normal urinary excretion rates of 17-hydroxy- and 17-ketosteroids. The adrenal lesions were localized by intravenous pyelography or sonography and by angiographic procedures, while radioscanning by 131I-cholesterol showed no uptake of activity. Unilateral adrenalectomy was performed in four cases. After appearance of hormonally active metastases o,p'DDD was administered as an antitumor agent in 3 patients. Two of these patients died 10 and 25 months after surgery. One patient with multiple lung metastases is still alive, with a postoperative course of more than 80 months.