title: Surgical Management of Carney Complex-Associated Pituitary Pathology.
abstract: BACKGROUND
  Carney complex (CNC) is a familial neoplasia syndrome that is associated with pituitary-associated hypersecretion of growth hormone (GH) (acromegaly). The underlying cause of pituitary GH hypersecretion and its management have been incompletely defined.
  
  
  OBJECTIVE
  To provide biological insight into CNC-associated pituitary pathology and improve management, we analyzed findings in CNC patients who underwent transsphenoidal surgery.
  
  
  METHODS
  Consecutive CNC patients at the National Institutes of Health with acromegaly and imaging evidence of a pituitary adenoma(s) who underwent transsphenoidal resection of tumor(s) were included. Prospectively acquired magnetic resonance imaging and biochemical, surgical, and histological data were analyzed.
  
  
  RESULTS
  Seven acromegalic CNC patients (2 male, 5 female) were included. The mean age at surgery was 29.7 years (range, 18-44 years). The mean follow-up was 4.7 years (range, 0.2-129 months). Magnetic resonance imaging revealed a single pituitary adenoma in 4 patients and multiple pituitary adenomas in 3 patients. Whereas patients with single discrete pituitary adenomas underwent selective adenomectomy, patients with multiple adenomas underwent selective adenomectomy of multiple tumors, as well as partial or total hypophysectomy. All adenomas were either GH and prolactin positive or exclusively prolactin positive. Pituitary tissue surrounding the adenomas in patients with multiple adenomas revealed hyperplastic GH- and prolactin-positive tissue.
  
  
  CONCLUSIONS
  CNC-associated acromegaly results from variable pituitary pathology, including a single GH-secreting adenoma or multiple GH-secreting adenomas and/or GH hypersecretion of the pituitary gland surrounding multiple adenomas. Although selective adenomectomy is the preferred treatment for cases of GH-secreting adenomas, multiple adenomas with associated pituitary gland GH hypersecretion may require partial or complete hypophysectomy to achieve biochemical remission.
authors: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.
authors: Russell R
authors: RR
authors: Lonser
authors: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.
authors: Gautam U
authors: GU
authors: Mehta
authors: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.
authors: Bogdan A
authors: BA
authors: Kindzelski
authors: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.
authors: Abhik
authors: A
authors: Ray-Chaudhury
authors: Neuropathology Pro-gram, Department of Pathology, Yale University School of Medicine, New Haven, Connecticut.
authors: Alexander O
authors: AO
authors: Vortmeyer
authors: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.
authors: Robert
authors: R
authors: Dickerman
authors: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.
authors: Edward H
authors: EH
authors: Oldfield
mesh_terms: D000172:Acromegaly / Q000000981:diagnostic imaging / Q000601:surgery*; D000236:Adenoma / Q000000981:diagnostic imaging / Q000601:surgery*; D000293:Adolescent; D000328:Adult; D056733:Carney Complex / Q000000981:diagnostic imaging / Q000601:surgery*; D019468:Disease Management; D005260:Female; D019382:Human Growth Hormone / Q000378:metabolism*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000009:adverse effects; D008297:Male; D010900:Pituitary Diseases / Q000000981:diagnostic imaging / Q000601:surgery*; D010902:Pituitary Gland / Q000000981:diagnostic imaging / Q000378:metabolism / Q000601:surgery; D010911:Pituitary Neoplasms / Q000000981:diagnostic imaging / Q000601:surgery*; D055815:Young Adult
chemical_list: D019382:Human Growth Hormone
keywords: Acromegaly; Adenoma; Carney complex; Pituitary; Surgery