title: Cushing's syndrome due to adrenocortical carcinoma - a comphrensive clinical and biochemical study of patients treated by surgery and chemotherapy.
abstract: Four post-menopausal women had Cushing's syndrome due to adrenal cortical carcinomas. Comprehensive analyses of blood and urinary steroids showed that although the steroid profiles differed between patients, the pattern in each patient remained almost constant as the disease progressed, or remitted due to therapy. Elevations of serum testosterone and oestradiol were commensurate with the extent of virilisation, and the urinary output of aldosterone was associated with the severity of hypertension. A new finding was that all had substantially increased urinary free deoxycorticosterone. Complete surgical removal of the primary tumours was impossible but when most of the tumour tissue was removed, full clinical and biochemical remissions were obtained for a short time in 2 patients. One patient obtained a clinical and biochemical remission from op'DDD. In another patient the drug caused reduction both in blood pressure and in urinary aldosterone excretion, but there were unpleasant side effects. A third patient could not tolerate op'DDD. Metyrapone therapy produced neither clinical nor biochemical improvement in 3 patients. The mean duration of survival was 17 months after the first symptoms and 10 months from the date of operation. Despite advances in drug therapy, adrenal cortical carcinoma remains a lethal disease. Biochemical screening of multiple steroids offers a means of early diagnosis and disease monitoring. Extensive surgical removal of the tumour offers the best chance of a clinical and biochemical remission.
mesh_terms: D015068:17-Ketosteroids / Q000652:urine; D000306:Adrenal Cortex Neoplasms / Q000150:complications* / Q000188:drug therapy / Q000601:surgery; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000450:Aldosterone / Q000652:urine; D002277:Carcinoma / Q000150:complications* / Q000188:drug therapy / Q000601:surgery; D003350:Cortodoxone / Q000097:blood; D003480:Cushing Syndrome / Q000209:etiology* / Q000378:metabolism; D003900:Desoxycorticosterone / Q000652:urine; D004573:Electrolytes / Q000097:blood; D004958:Estradiol / Q000097:blood; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007986:Luteinizing Hormone / Q000097:blood; D008797:Metyrapone / Q000627:therapeutic use*; D008875:Middle Aged; D008939:Mitotane / Q000009:adverse effects / Q000627:therapeutic use*; D011279:Pregnanetriol / Q000652:urine; D013739:Testosterone / Q000097:blood
chemical_list: D015068:17-Ketosteroids; D004573:Electrolytes; D011279:Pregnanetriol; D013739:Testosterone; D003900:Desoxycorticosterone; D000450:Aldosterone; D004958:Estradiol; D008939:Mitotane; D000324:Adrenocorticotropic Hormone; D007986:Luteinizing Hormone; D003350:Cortodoxone; D006854:Hydrocortisone; D008797:Metyrapone