[Cushing's disease in the 90's: a review]

Abstract

Since 1932 when Cushing's disease has been described, several important advances have been made in diagnosis and management. However several points remain obscure and there is no general agreement among authors. An accurate biological diagnosis is one of the difficulties found when we face a patient with hypercortisolism. In addition, corticotropin dependent syndrome should be distinguished from the independent one, as well as identifying the source of ACTH hypersecretion. The main problem in Cushing's disease is to localise the secreting adenoma and at the moment there is not any diagnostic method with absolute sensibility and specificity. Magnetic Resonance Imaging shows a sensibility of 77% and 87% especificity; inferior petrosal sinus sampling may help in localising the side, where the adenoma lies, although an appropriate technique is mandatory. New developing techniques, such as intraoperative doppler and assessment of ACTH levels in the peripituitary veins may help us in localising the source of ACTH hypersecretion. Sometimes the adenoma cannot be identified and a surgical exploration of the pituitary is required. If an adenoma is found, a 89% probility of curation has been reported; on the contrary, incomplete tumor removal, no tumor found at surgery, or the presence of the so-called corticotrop hyperplasia where followed of bad results and may explain the failed surgery. Then, hypofisectomy, hemi-hypofisectomy, radiotherapy, photon knife or gamma knife, may be of help in the control of this condition. We reviewed the recent literature and analysed the diagnostic strategies and teatments currently available for this illness. In addition we propose an algohritm for diagnosis and treatment and analyze our results in a consecutive series of 38 patients.