McCune-Albright syndrome

Last revised by Subhan Iqbal on 13 Feb 2022

Citation, DOI, disclosures and article data

Citation:

Gaillard F, Iqbal S, Matic M, et al. McCune-Albright syndrome. Reference article, Radiopaedia.org (Accessed on 03 Dec 2023) https://doi.org/10.53347/rID-6568

DOI:

https://doi.org/10.53347/rID-6568

Permalink:

https://radiopaedia.org/articles/6568

rID:

6568

Article created:

14 Jul 2009, Frank Gaillard ◈

Disclosures:

At the time the article was created Frank Gaillard had no recorded disclosures.

View Frank Gaillard's current disclosures

Last revised:

13 Feb 2022, Subhan Iqbal

Disclosures:

At the time the article was last revised Subhan Iqbal had no recorded disclosures.

View Subhan Iqbal's current disclosures

Revisions:

14 times, by 12 contributors - see full revision history and disclosures

Systems:

Musculoskeletal, Urogenital

Sections:

Syndromes

Tags:

endocrine

Synonyms:

McCune-Albright-Sternberg syndrome

McCune-Albright syndrome (MAS) (also known as McCune-Albright-Sternberg syndrome) is a genetic disorder characterized by the association of:

endocrinopathy: precocious puberty
polyostotic fibrous dysplasia: more severe than in sporadic cases
cutaneous pigmentation: coast of Maine 'café au lait' spots

leg pain, limp, pathological fracture: ~ 75%
abnormal vaginal bleeding: ~25%
coast of Maine café au lait spots
- predominantly on posterior trunk: ~40% (range 30-50%)
- often ipsilateral to bone lesions
- irregular borders
hypercortisolism (Cushing syndrome): ~7% ³
hyperthyroidism ⁵
phosphate wasting

Pathology

Genetics

MAS results from a sporadically occurring somatic GNAS gene mutation ².

History and etymology

The condition is named after:

Fuller Albright (1900-1969), an American endocrinologist
Donovan James McCune (1902-1976), an American pediatrician
Carl Sternberg (1872-1935), an Austrian pathologist

Radiographic features

Role of imaging in MAS is predominantly to identify and define fibrous dysplasia (refer to the article for specific features). It is also complementary in identifying related endocrinological conditions.