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%