McCune-Albright syndrome

Bone
Fibrous and Fibroosseous tumors of bone
McCune-Albright syndrome

Author: Nat Pernick, M.D. (see Authors page)

Revised: 7 October 2016, last major update August 2013

Copyright: (c) 2003-2016, PathologyOutlines.com, Inc.

PubMed Search: Fibrous dysplasia mccune-albright syndrome

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

Table of Contents

Definition / general | Gross images | Additional references

Cite this page: McCune-Albright syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/bonefibrousdysplasiamccunealbright.html. Accessed July 15th, 2017.

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:

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

Additional references

eMedicine - McCune Albright Syndrome