Treating Nelson's Syndrome after BLA

Sustained improvements in plasma ACTH and clinical status in a patient with Nelson's syndrome treated with pasireotide LAR, a multireceptor somatostatin analog

Laurence Katznelson, MD
Stanford School of Medicine, Departments of Neurosurgery and Medicine, 875 Blake Wilbur Dr. MC 5821, Stanford, CA 94305-5821

Address all correspondence and requests for reprints to: Laurence Katznelson, MD, Stanford School of Medicine, Departments of Neurosurgery and Medicine, 875 Blake Wilbur Dr. MC 5821, Stanford, CA 94305-5821, lkatznelson@stanford.edu, tel: (650) 721-1020, fax: (650) 736-8100.

• Published online before print March 28, 2013, doi:10.1210/jc.2013-1497 The Journal of Clinical Endocrinology & Metabolism

http://m.jcem.endojournals.org/content/early/2013/03/28/jc.2013-1497.abstract

Abstract

Context: Nelson's syndrome refers to aggressive pituitary corticotroph adenoma growth after bilateral adrenalectomy (BLA) for treatment of Cushing's disease (CD). Pasireotide, a novel somatostatin analog, has been effective in treating CD. Here, the first case report of a patient with Nelson's syndrome treated with pasireotide is presented.

Case Presentation: A 55 yo female was diagnosed with CD in 1973 at age 15 y and underwent BLA 1 year later. She subsequently developed Nelson's syndrome and underwent multiple surgeries, and radiotherapy for adenoma growth.

Following presentation with ocular pain, third cranial nerve palsy, and finding of suprasellar tumor enlargement with hemorrhage, she began pasireotide LAR 60 mg/28 days IM. At baseline, fasting plasma ACTH was 42,710 pg/mL (normal, 5–27) and fasting plasma glucose was 98 mg/dL. After 1 month, ACTH declined to 4,272 pg/mL and has remained stable over 19 months of follow up. Hyperpigmentation progressively improved. MRI scans show reduction in the suprasellar component. Fasting plasma glucose increased to 124 mg/dL and she underwent diabetes management.

Evidence Acquisition and Synthesis:In this clinical case seminar, the current understanding of the treatment of Nelson's syndrome, including use of pasireotide in Cushing's disease, are summarized.

Conclusion: A case of Nelson's syndrome with clinically significant and dramatic biochemical and clinical responses to pasireotide administration is reported. Hyperglycemia was noted following pasireotide administration. Pasireotide may represent a useful tool in the medical management of Nelson's syndrome. Further study of the potential benefits and risks of pasireotide in this population is necessary.

Received February 22, 2013.
Accepted March 25, 2013.
Copyright © 2013 by The Endocrine Society