title: Sustained improvements in plasma ACTH and clinical status in a patient with Nelson's syndrome treated with pasireotide LAR, a multireceptor somatostatin analog.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  METHODS
  In this clinical case seminar, the current understanding of the treatment of Nelson's syndrome and the use of pasireotide in CD are summarized.
  
  
  CONCLUSIONS
  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.affiliation: Stanford School of Medicine, Department of Neurosurgery, 875 Blake Wilbur Drive MC 5821, Stanford, California 94305-5821, USA. lkatznelson@stanford.edu
authors.forename: Laurence
authors.initials: L
authors.lastname: Katznelson
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000097:blood*; D020863:Central Nervous System Cysts / Q000209:etiology / Q000517:prevention & control; D003692:Delayed-Action Preparations; D054873:Dipeptidyl-Peptidase IV Inhibitors / Q000627:therapeutic use; D005260:Female; D013007:Growth Hormone-Releasing Hormone / Q000037:antagonists & inhibitors; D006801:Humans; D006943:Hyperglycemia / Q000139:chemically induced / Q000188:drug therapy; D017495:Hyperpigmentation / Q000209:etiology / Q000517:prevention & control; D008875:Middle Aged; D009347:Nelson Syndrome / Q000097:blood / Q000188:drug therapy* / Q000503:physiopathology; D011719:Pyrazines / Q000627:therapeutic use; D012720:Severity of Illness Index; D000068900:Sitagliptin Phosphate; D013004:Somatostatin / Q000008:administration & dosage / Q000009:adverse effects / Q000031:analogs & derivatives* / Q000627:therapeutic use; D016896:Treatment Outcome; D014230:Triazoles / Q000627:therapeutic use
chemical_list: D003692:Delayed-Action Preparations; D054873:Dipeptidyl-Peptidase IV Inhibitors; D011719:Pyrazines; D014230:Triazoles; D013004:Somatostatin; D000324:Adrenocorticotropic Hormone; D013007:Growth Hormone-Releasing Hormone; C517782:pasireotide; D000068900:Sitagliptin Phosphate