MAS: Classic triad of polyostotic fibrous dysplasia (FD), hyperfunctioning endocrinopathies (precocious puberty most common, especially in girls), and cutaneous hyperpigmentation (café au lait skin pigmentation)
New recommendations: MAS diagnosis defined as combination of FD and 1 or more extraskeletal features or presence of 2 or more extraskeletal features
Do not require FD for diagnosis of MAS to reflect improved understanding of molecular pathogenesis of MAS
FD: Normal medullary bone replaced by mixture of fibrous tissue and immature, weak, woven bone
Defect in osteoblastic differentiation and maturation
One of most common fibroosseous lesions
IMAGING
General Features
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Javaid MK et al: Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome: a consensus statement from the FD/MAS international consortium. Orphanet J Rare Dis. 14(1):139, 2019
Turan S et al: GNAS spectrum of disorders. Curr Osteoporos Rep. 13(3):146-58, 2015
Frisch CD et al: Fibrous dysplasia of the temporal bone: A review of 66 cases. Laryngoscope. ePub, 2014
Brown RJ et al: Cushing syndrome in the McCune-Albright syndrome. J Clin Endocrinol Metab. 95(4):1508-15, 2010
Ferreira EC et al: Whole-body MR imaging for the evaluation of McCune-albright syndrome. J Magn Reson Imaging. 31(3):706-10, 2010
Chen Y et al: Craniofacial fibrous dysplasia associated with McCune-Albright syndrome. J Oral Maxillofac Surg. 67(3):637-44, 2009
Godse AS et al: Fibrous dysplasia of the maxilla. J Pediatr Surg. 44(4):849-51, 2009
Medina YN et al: Evolving diagnosis of McCune-Albright syndrome. atypical presentation and follow up. J Pediatr Endocrinol Metab. 22(4):373-7, 2009
Rahman AM et al: Craniofacial fibrous dysplasia: clinical characteristics and long-term outcomes. Eye (Lond). 23(12):2175-81, 2009
Saxon PH et al: McCune-Albright Syndrome: intensely hypermetabolic polyostotic fibrous dysplasia on F-18 FDG-PET. Clin Nucl Med. 34(11):795-7, 2009
Berrebi O et al: F-18 fluorodeoxyglucose (FDG) PET in the diagnosis of malignant transformation of fibrous dysplasia in the pelvic bones. Clin Nucl Med. 33(7):469-71, 2008
Bulakbaşi N et al: CT and MRI in the evaluation of craniospinal involvement with polyostotic fibrous dysplasia in McCune-Albright syndrome. Diagn Interv Radiol. 14(4):177-81, 2008
Chapurlat RD et al: Fibrous dysplasia of bone and McCune-Albright syndrome. Best Pract Res Clin Rheumatol. 22(1):55-69, 2008
Daneman A et al: McCune-Albright syndrome. J Pediatr Endocrinol Metab. 20(12):1265, 2007
Defilippi C et al: Image diagnosis in McCune-Albright syndrome. J Pediatr Endocrinol Metab. 19 Suppl 2:561-70, 2006
Leet AI et al: Fibrous dysplasia in the spine: prevalence of lesions and association with scoliosis. J Bone Joint Surg Am. 86-A(3):531-7, 2004
Kaushik S et al: Malignant transformation of fibrous dysplasia into chondroblastic osteosarcoma. Skeletal Radiol. 31(2):103-6, 2002
Sener RN: An extensive type of polyostotic fibrous dysplasia. Pediatr Radiol. 27(4):339-41, 1997
Sisayan R et al: Polyostotic fibrous dysplasia in McCune-Albright syndrome diagnosed by bone scintigraphy. Clin Nucl Med. 22(6):410-2, 1997
Carod J et al: [Neuroimaging, CT and MR brain findings in a case of McCune-Albright syndrome.] Rev Neurol. 24(132):979-81, 1996
Inamo Y et al: Findings on magnetic resonance imaging of the spine and femur in a case of McCune-Albright syndrome. Pediatr Radiol. 23(1):15-8, 1993
Related Anatomy
Loading...
Related Differential Diagnoses
Loading...
References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
TERMINOLOGY
Abbreviations
McCune-Albright syndrome (MAS)
Definitions
MAS: Classic triad of polyostotic fibrous dysplasia (FD), hyperfunctioning endocrinopathies (precocious puberty most common, especially in girls), and cutaneous hyperpigmentation (café au lait skin pigmentation)
New recommendations: MAS diagnosis defined as combination of FD and 1 or more extraskeletal features or presence of 2 or more extraskeletal features
Do not require FD for diagnosis of MAS to reflect improved understanding of molecular pathogenesis of MAS
FD: Normal medullary bone replaced by mixture of fibrous tissue and immature, weak, woven bone
Defect in osteoblastic differentiation and maturation
One of most common fibroosseous lesions
IMAGING
General Features
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Javaid MK et al: Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome: a consensus statement from the FD/MAS international consortium. Orphanet J Rare Dis. 14(1):139, 2019
Turan S et al: GNAS spectrum of disorders. Curr Osteoporos Rep. 13(3):146-58, 2015
Frisch CD et al: Fibrous dysplasia of the temporal bone: A review of 66 cases. Laryngoscope. ePub, 2014
Brown RJ et al: Cushing syndrome in the McCune-Albright syndrome. J Clin Endocrinol Metab. 95(4):1508-15, 2010
Ferreira EC et al: Whole-body MR imaging for the evaluation of McCune-albright syndrome. J Magn Reson Imaging. 31(3):706-10, 2010
Chen Y et al: Craniofacial fibrous dysplasia associated with McCune-Albright syndrome. J Oral Maxillofac Surg. 67(3):637-44, 2009
Godse AS et al: Fibrous dysplasia of the maxilla. J Pediatr Surg. 44(4):849-51, 2009
Medina YN et al: Evolving diagnosis of McCune-Albright syndrome. atypical presentation and follow up. J Pediatr Endocrinol Metab. 22(4):373-7, 2009
Rahman AM et al: Craniofacial fibrous dysplasia: clinical characteristics and long-term outcomes. Eye (Lond). 23(12):2175-81, 2009
Saxon PH et al: McCune-Albright Syndrome: intensely hypermetabolic polyostotic fibrous dysplasia on F-18 FDG-PET. Clin Nucl Med. 34(11):795-7, 2009
Berrebi O et al: F-18 fluorodeoxyglucose (FDG) PET in the diagnosis of malignant transformation of fibrous dysplasia in the pelvic bones. Clin Nucl Med. 33(7):469-71, 2008
Bulakbaşi N et al: CT and MRI in the evaluation of craniospinal involvement with polyostotic fibrous dysplasia in McCune-Albright syndrome. Diagn Interv Radiol. 14(4):177-81, 2008
Chapurlat RD et al: Fibrous dysplasia of bone and McCune-Albright syndrome. Best Pract Res Clin Rheumatol. 22(1):55-69, 2008
Daneman A et al: McCune-Albright syndrome. J Pediatr Endocrinol Metab. 20(12):1265, 2007
Defilippi C et al: Image diagnosis in McCune-Albright syndrome. J Pediatr Endocrinol Metab. 19 Suppl 2:561-70, 2006
Leet AI et al: Fibrous dysplasia in the spine: prevalence of lesions and association with scoliosis. J Bone Joint Surg Am. 86-A(3):531-7, 2004
Kaushik S et al: Malignant transformation of fibrous dysplasia into chondroblastic osteosarcoma. Skeletal Radiol. 31(2):103-6, 2002
Sener RN: An extensive type of polyostotic fibrous dysplasia. Pediatr Radiol. 27(4):339-41, 1997
Sisayan R et al: Polyostotic fibrous dysplasia in McCune-Albright syndrome diagnosed by bone scintigraphy. Clin Nucl Med. 22(6):410-2, 1997
Carod J et al: [Neuroimaging, CT and MR brain findings in a case of McCune-Albright syndrome.] Rev Neurol. 24(132):979-81, 1996
Inamo Y et al: Findings on magnetic resonance imaging of the spine and femur in a case of McCune-Albright syndrome. Pediatr Radiol. 23(1):15-8, 1993
STATdx includes over 200,000 searchable images, including x-ray, CT, MR, and ultrasound images. To access all images, please log in or subscribe.