title: Nelson's syndrome after bilateral adrenalectomy for Cushing's disease.
abstract: BACKGROUND
  Presentation and outcome of Nelson's syndrome after bilateral adrenalectomy is variable.
  
  
  METHODS
  Clinical records of 39 patients who underwent bilateral adrenalectomy for primary or recurrent Cushing's disease during a 15-year period were analyzed for frequency and evolution of Nelson's syndrome.
  
  
  RESULTS
  The study included 32 females and 7 males with a mean age of 31 years; 20 patients had a hypophysectomy as the initial procedure, and 19 had an adrenalectomy. Of the group, 17 patients received prophylactic radiation therapy to the pituitary gland. A total of 11 patients, none of whom had received prophylactic radiation therapy, developed Nelson's syndrome (determined by skin hyperpigmentation, elevated serum ACTH levels, and enlargement of a previous pituitary tumor or development of a new tumor in patients with no previous pituitary abnormality) over a mean follow-up period of 53 months. Treatment for Nelson's syndrome included valproic acid, radiation therapy, and hypophysectomy as monotherapy or combined therapy. Of the remaining 28 patients, 10 (7 without prophylactic radio therapy) developed skin hyperpigmentation and increased ACTH levels without a tumor.
  
  
  CONCLUSIONS
  Nelson's syndrome is a frequent complication after bilateral adrenalectomy in the absence of prophylactic radiotherapy (28%). The syndrome can be successfully controlled by medical treatment and or radiotherapy; patients rarely require hypophysectomy.
authors.affiliation: Department of Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
authors.forename: Alejandra
authors.initials: A
authors.lastname: Gil-Cárdenas
authors.forename: Miguel F
authors.initials: MF
authors.lastname: Herrera
authors.forename: Araceli
authors.initials: A
authors.lastname: Díaz-Polanco
authors.forename: Juan Manuel
authors.initials: JM
authors.lastname: Rios
authors.forename: Juan Pablo
authors.initials: JP
authors.lastname: Pantoja
mesh_terms: D000293:Adolescent; D000315:Adrenalectomy / Q000009:adverse effects*; D000328:Adult; D003480:Cushing Syndrome / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009347:Nelson Syndrome / Q000453:epidemiology / Q000209:etiology*; D015995:Prevalence