Bone
Fibrous and Fibroosseous tumors of bone
McCune-Albright syndrome



Topic Completed: 1 August 2013

Revised: 16 May 2019

Copyright: 2003-2016, PathologyOutlines.com, Inc.

PubMed Search: Fibrous dysplasia mccune-albright syndrome

Related topics: Fibrous dysplasia, Monostotic, Polyostotic, Mazabraud syndrome, Fibrous dysplasia protuberans



Nat Pernick, M.D.
Page views in 2018: 472
Page views in 2019 to date: 255
Cite this page: Pernick N. McCune-Albright syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/bonefibrousdysplasiamccunealbright.html. Accessed June 17th, 2019.
Definition / general
  • 1 - 3% of cases
  • Common presentation is precocious puberty in girls
  • Due to somatic mutation of c-fos oncogene (alpha subunit of signal-transducing G proteins - Gsalpha) that causes activation of cAMP pathway
  • Polyostotic fibrous dysplasia, cafe-au-lait skin pigmentation (large, dark lesions with serpiginous [“coast of Maine”] borders in chest, neck, back), almost exclusively in women
  • Also endocrine abnormalities (precocious puberty in girls, hyperthyroidism, pituitary adenomas that secrete growth hormone, primary adrenal hyperplasia)
  • Malignant transformation in 4%
Gross images

Images hosted on other servers:
Missing Image

4 year old girl with mosaic pattern
of darkened skin and body cast
due to fractures

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