FACTORS INFLUENCING THE IMMEDIATE AND LATE OUTCOME OF CUSHINGS-DISEASE TREATED BY TRANSSPHENOIDAL SURGERY - A RETROSPECTIVE STUDY BY THE EUROPEAN CUSHINGS-DISEASE SURVEY GROUP

Hypercortisolism attributable to hypersecretion of ACTH by a pituitary adenoma is an uncommon and progressively lethal disease. Because of i ts rarity, it has been difficult to collect a large series of patients in order to identify the prognostic factors influencing the outcome a fter transsphenoidal surgery. We conducted a multicenter, retrospectiv e analysis of the early and late results of surgical treatment of Cush ing's disease. Files of patients with Cushing's disease who underwent transsphenoidal surgery between 1975 and 1990 were collected from 25 i nstitutions throughout Europe. Data from 668 of 716 patients were suit able for statistical analyses. Surgical mortality was 1.9%, and major morbidity occurred in 97 patients (14.5%). Clinical and biochemical re mission of Cushing's disease after surgery occurred in 510 cases (76.3 %). Identification of the tumor by neuroradiological imaging or at ope ration with histopathological corroboration was associated with remiss ion of hypercortisolism. Recurrence of the disease occurred in 65 (12. 7%) of 510 patients in remission after surgery at a mean time of 39.3 months (range 6-104 months). The distribution of the recurrences did n ot show any apparent plateau or cluster throughout the follow-up perio d. Low postoperative steroid levels, absence of cortisol response to C RH, and the need for long-term glucocorticoid substitution therapy wer e all associated with a high probability of long-term remission. Our s tudy demonstrates that transsphenoidal surgery is a safe and effective treatment for patients with Cushing's disease. However, after success ful surgery there is a steady increase in the percentage of recurrence s, which continues with time. Patients who after operation had hypoadr enocorticism and needed long-term glucocorticoid substitution therapy had the lowest risk of relapse.