FAILURE OF PARTIAL HYPOPHYSECTOMY AS DEFINITIVE TREATMENT IN CUSHINGS-DISEASE OWING TO NODULAR CORTICOTROPE HYPERPLASIA - REPORT OF 4 CASES

Nodular corticotrope hyperplasia is a rare pathology causing Gushing's syndrome owing to a primary pituitary disease or ectopic CRH producti on. In this study, we evaluated the laboratory and pathological findin gs and results of transsphenoidal pituitary surgery in four patients w ith Gushing's disease. Dynamic tests of pituitary-adrenal function (de xamethasone suppression, metyrapone, CRH, and DDAVP tests) were done b efore and after transsphenoidal pituitary surgery. Plasma and total ur inary cortisol, serum 11-deoxycortisol, and plasma ACTH were determine d by RIA. Hormonal dynamic tests and radiologic studies were compatibl e with a pituitary ACTH source. The transsphenoidal surgery revealed t he presence of corticotrope hyperplasia confirmed by immunoperoxidase stain and a preserved reticulum framework in the removed pituitary tis sue of these four patients. The pituitary surgery led to a short perio d of improvement in two of the patients (1 and 4), a 3-yr remission in one patient (patient 2), and no improvement in one (patient 3). We co nclude that although our patients appear to have inadequate suppressio n with high-dose dexamethasone, there is no way to diagnose this patho logy presurgically, and that total hypophysectomy, bilateral adrenalec tomy, and irradiation are the only alternatives for definitive treatme nt. A CRH-secreting ectopic tumor could not be found in our patients e ither before or after surgery in the follow-up period.