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

Abstract

CONTEXT:

Nelson's syndrome refers to aggressive pituitary corticotroph adenoma growth after bilateral adrenalectomy 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-year-old female was diagnosed with CD in 1973 at age 15 years and underwent bilateral adrenalectomy 1 year later. She subsequently developed Nelson's syndrome and underwent multiple surgeries and radiotherapy for adenoma growth. After presentation with ocular pain, third cranial nerve palsy, and a finding of suprasellar tumor enlargement with hemorrhage, she began pasireotide long-acting release 60 mg/28 days im. At baseline, fasting plasma ACTH was 42 710 pg/mL (normal, 5-27 pg/mL), and fasting plasma glucose was 98 mg/dL. After 1 month, ACTH declined to 4272 pg/mL, and it has remained stable over 19 months of follow-up. Hyperpigmentation progressively improved. Magnetic resonance imaging scans show reduction in the suprasellar component. Fasting plasma glucose increased to 124 mg/dL, and the patient underwent diabetes management.

EVIDENCE ACQUISITION AND SYNTHESIS:

In this clinical case seminar, the current understanding of the treatment of Nelson's syndrome and the use of pasireotide in CD 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 after 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.

• Authors: Laurence Katznelson
• Journal: The Journal of clinical endocrinology and metabolism (J Clin Endocrinol Metab) Vol. 98 Issue 5 Pg. 1803-7 (May 2013) ISSN: 1945-7197 [Electronic] United States
• PMID: 23539733 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Delayed-Action Preparations
• Dipeptidyl-Peptidase IV Inhibitors
• Pyrazines
• Triazoles
• Somatostatin
• Adrenocorticotropic Hormone
• Growth Hormone-Releasing Hormone
• pasireotide
• Sitagliptin Phosphate

Topics

• Adrenocorticotropic Hormone (blood)
• Central Nervous System Cysts (etiology, prevention & control)
• Delayed-Action Preparations
• Dipeptidyl-Peptidase IV Inhibitors (therapeutic use)
• Female
• Growth Hormone-Releasing Hormone (antagonists & inhibitors)
• Humans
• Hyperglycemia (chemically induced, drug therapy)
• Hyperpigmentation (etiology, prevention & control)
• Middle Aged
• Nelson Syndrome (blood, drug therapy, physiopathology)
• Pyrazines (therapeutic use)
• Severity of Illness Index
• Sitagliptin Phosphate
• Somatostatin (administration & dosage, adverse effects, analogs & derivatives, therapeutic use)
• Treatment Outcome
• Triazoles (therapeutic use)