NELSON'S SYNDROME: INCIDENCE AND PROGNOSIS

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Abstract
Fifty patients bilaterally adrenalectomized for Cushing''s disease were followed for 1-22 (mean, 12) yr. In 14 of them (28%), Nelson''s syndrome appeared within 1.5-12 (mean, 4.8) yr after adrenalectomy. All the patients were deeply pigmented, 12 had a radiologically demonstrable tumor and 6 had visual defects. Plasma ACTH levels ranged from 450-8000 ng/l. In every case at least one estimation during circadian studies equalled 2000 ng/l. One patient with an anaplastic pituitary tumor died 3 yr after the discovery of the tumor. Anaplasia was also diagnosed in another patient with recurrence of pituitary tumor after a transsphenoidal operation. Symptoms of tumor infarction occurred in 2 patients, followed by clinical remission of Nelson''s syndrome. In the majority of cases, the course was benign. All cases, should evidently be followed indefinitely because Nelson''s tumors are not infrequently aggressive.