A Long-Term Study of the Treatment of Nelson's Syndrome With Gamma Knife Radiosurgery

• Published in: Neurosurgery Vol. 83; no. 3; pp. 430 - 436
• Main Authors: Caruso, James P, Patibandla, Mohana Rao, Xu, Zhiyuan, Vance, Mary Lee, Sheehan, Jason P
• Format: Journal Article
• Language: English
• Published: United States Oxford University Press 01.09.2018; Copyright by the Congress of Neurological Surgeons; Wolters Kluwer Health, Inc
• Subjects: Cushing syndrome; Medical records; Neurosurgery; Patients; Pituitary gland; Radiation therapy; Regression analysis; Surgery; Surgical techniques; Tumors; Bilateral adrenalectomy; Nelson's syndrome; Cushing's disease; Pituitary adenoma; Gamma Knife radiosurgery; radiosurgery; Stereotactic Radiosurgery
• ISSN: 0148-396X; 1524-4040
• DOI: 10.1093/neuros/nyx426

Abstract BACKGROUND Nelson's syndrome may be a devastating complication for patients with Cushing's disease who underwent a bilateral adrenalectomy. Previous studies have demonstrated that stereotactic radiosurgery (SRS) can be used to treat patients with Nelson's syndrome. OBJECTIVE To report a retrospective study of patients with Nelson's syndrome treated with Gamma Knife radiosurgery to evaluate the effect of SRS on endocrine remission and tumor control. METHODS Twenty-seven patients with Nelson's syndrome treated with Gamma Knife radiosurgery after bilateral adrenalectomy were included in this study. After radiosurgery, patients were followed with serial adrenocorticotropic hormone (ACTH) levels and MRI sequences to assess for endocrine remission and tumor control. Cox proportional hazards regression analysis was used to evaluate the relationship between the time to remission and potential prognostic factors. RESULTS In 21 patients with elevated ACTH prior to SRS and endocrine follow-up data, 14 (67%) had decreased or stable ACTH levels, and 7 achieved a normal ACTH level at a median of 115 mo (range 7-272) post-SRS. Tumor volume was stable or reduced after SRS in 92.5% of patients (25/27) with radiological follow-up. Time to remission was not significantly associated with the ACTH prior to SRS (P = .252) or with the margin dose (P = .3). However, a shorter duration between the patient's immediate prior transsphenoidal resection and SRS was significantly associated with a shorter time to remission (P = .045). CONCLUSION This retrospective analysis suggests that SRS is an effective means of achieving endocrine remission and tumor control in patients with Nelson's syndrome.