Results of treatment in 320 patients with Cushing's syndrome in Japan (Japanese)

Abstract

In 1972 data on 320 patients with Cushing's syndrome were collected from 37 university hospitals and 1 affiliated hospital through questionnaires sent out to them. The data comprised all cases of Cushing's syndrome encountered in each medical center for the 10 years prior to, and including 1972. 113 cases (35.3%) of adenoma, 187 cases (58.4%) of hyperplasia, 16 cases (5.0%) of cancer and 4 cases (1.3%) of ectopic ACTH syndrome comprise the present series. Age and sex distributions of Cushing's syndrome due to adenoma and hyperplasia revealed a definite preponderance of females ranging in age from 20 to 40 years. Over 90% of patients with adenoma were cured by surgical removal of the tumor, compatible with the previously published series and no recurrence was reported. Pituitary irradiation was performed in 65 cases (35%) of hyperplasia as initial therapy or combined therapy with adrenal surgery. Though reserpine was concomitantly administered to a considerable number of cases treated by pituitary irradiation, favourable response was observed in only about 30% of these cases due to hyperplasia with pituitary irradiation. Excellent results (effective in over 90% of cases) were obtained in 53 patients with hyperplasia subjected to bilateral total adrenalectomy, as initial therapy or when other treatments were unsuccessful. Subtotal adrenalectomy or unilateral adrenalectomy revealed much less favourable results: effective results were obtained in around 60% of cases treated by subtotal adrenalectomy and in only 30% of cases treated by unilateral adrenalectomy. Therapeutic results of patients with hyperplasia treated by subtotal or unilateral adrenalectomy combined with pituitary irradiation surpassed those treated by adrenal surgery alone. In the present series, the main sequence of management was subtotal or unilateral adrenalectomy followed by pituitary irradiation or pituitary irradiation followed by subtotal or unilateral adrenalectomy, when either procedure as initial therapy was unsuccessful. This is in marked contrast with the mode of treatment abroad, where adrenal surgery is generally limited to bilateral total adrenalectomy. Nelson syndrome developed in 2 patients after bilateral total adrenalectomy and in 1 patient after subtotal adrenalectomy combined with pituitary irradiation. Also 5 patients showed increased skin pigmentation after bilateral total adrenalectomy. Cushing's syndrome recurred in 17 cases of hyperplasia, viz. in 4 cases after unilateral adrenalectomy, in 1 case after unilateral adrenalectomy combined with pituitary irradiation, in 10 cases after subtotal adrenalectomy and in 2 cases after pituitary irradiation. Apart from Cushing's syndrome caused by malignant neoplasm, 27 cases (adenoma 7 cases, hyperplasia 20 cases) were reported to have died. The mortality rate was 9%. The main causes of death were adrenal insufficiency, cerebrovascular accident and infection. On this occasion, opinions concerning the future management of Cushing's syndrome due to hyperplasia were obtained from physicians and surgeons. There are now very few who support treatment by pituitary irradiation, subtotal adrenalectomy or unilateral adrenalectomy alone. But many prefer bilateral total adrenalectomy combined with or without pituitary irradiation or a combination of subtotal adrenalectomy with pituitary irradiation. Apparently this may be ascribed to the poor results obtained by subtotal or unilateral adrenalectomy as mentioned above. It is notable that adrenal surgery combined with pituitary irradiation is recommended by physicians rather than by surgeons.