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McCune-Albright Syndrome
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        TERMINOLOGY

        • Abbreviations

          • McCune-Albright syndrome (MAS)
        • Definitions

          • MAS: Subtype of polyostotic fibrous dysplasia (FD)
            • Classic triad of polyostotic FD, endocrine dysfunction (precocious puberty most common), and cutaneous hyperpigmentation (café au lait skin pigmentation)
          • Fibrous dysplasia (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

                              1. Turan S et al: GNAS spectrum of disorders. Curr Osteoporos Rep. 13(3):146-58, 2015
                              2. Frisch CD et al: Fibrous dysplasia of the temporal bone: A review of 66 cases. Laryngoscope. ePub, 2014
                              3. Brown RJ et al: Cushing syndrome in the McCune-Albright syndrome. J Clin Endocrinol Metab. 95(4):1508-15, 2010
                              4. Ferreira EC et al: Whole-body MR imaging for the evaluation of McCune-albright syndrome. J Magn Reson Imaging. 31(3):706-10, 2010
                              5. Chen Y et al: Craniofacial fibrous dysplasia associated with McCune-Albright syndrome. J Oral Maxillofac Surg. 67(3):637-44, 2009
                              6. Godse AS et al: Fibrous dysplasia of the maxilla. J Pediatr Surg. 44(4):849-51, 2009
                              7. Medina YN et al: Evolving diagnosis of McCune-Albright syndrome. atypical presentation and follow up. J Pediatr Endocrinol Metab. 22(4):373-7, 2009
                              8. Rahman AM et al: Craniofacial fibrous dysplasia: clinical characteristics and long-term outcomes. Eye (Lond). 23(12):2175-81, 2009
                              9. 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
                              10. 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
                              11. 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
                              12. Chapurlat RD et al: Fibrous dysplasia of bone and McCune-Albright syndrome. Best Pract Res Clin Rheumatol. 22(1):55-69, 2008
                              13. Daneman A et al: McCune-Albright syndrome. J Pediatr Endocrinol Metab. 20(12):1265, 2007
                              14. Defilippi C et al: Image diagnosis in McCune-Albright syndrome. J Pediatr Endocrinol Metab. 19 Suppl 2:561-70, 2006
                              15. 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
                              16. Kaushik S et al: Malignant transformation of fibrous dysplasia into chondroblastic osteosarcoma. Skeletal Radiol. 31(2):103-6, 2002
                              17. Sener RN: An extensive type of polyostotic fibrous dysplasia. Pediatr Radiol. 27(4):339-41, 1997
                              18. Sisayan R et al: Polyostotic fibrous dysplasia in McCune-Albright syndrome diagnosed by bone scintigraphy. Clin Nucl Med. 22(6):410-2, 1997
                              19. Carod J et al: [Neuroimaging, CT and MR brain findings in a case of McCune-Albright syndrome.] Rev Neurol. 24(132):979-81, 1996
                              20. 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
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                              Related Differential Diagnoses
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                              References
                              Tables

                              Tables

                              KEY FACTS

                              • Terminology

                                • Imaging

                                  • Top Differential Diagnoses

                                    TERMINOLOGY

                                    • Abbreviations

                                      • McCune-Albright syndrome (MAS)
                                    • Definitions

                                      • MAS: Subtype of polyostotic fibrous dysplasia (FD)
                                        • Classic triad of polyostotic FD, endocrine dysfunction (precocious puberty most common), and cutaneous hyperpigmentation (café au lait skin pigmentation)
                                      • Fibrous dysplasia (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

                                                          1. Turan S et al: GNAS spectrum of disorders. Curr Osteoporos Rep. 13(3):146-58, 2015
                                                          2. Frisch CD et al: Fibrous dysplasia of the temporal bone: A review of 66 cases. Laryngoscope. ePub, 2014
                                                          3. Brown RJ et al: Cushing syndrome in the McCune-Albright syndrome. J Clin Endocrinol Metab. 95(4):1508-15, 2010
                                                          4. Ferreira EC et al: Whole-body MR imaging for the evaluation of McCune-albright syndrome. J Magn Reson Imaging. 31(3):706-10, 2010
                                                          5. Chen Y et al: Craniofacial fibrous dysplasia associated with McCune-Albright syndrome. J Oral Maxillofac Surg. 67(3):637-44, 2009
                                                          6. Godse AS et al: Fibrous dysplasia of the maxilla. J Pediatr Surg. 44(4):849-51, 2009
                                                          7. Medina YN et al: Evolving diagnosis of McCune-Albright syndrome. atypical presentation and follow up. J Pediatr Endocrinol Metab. 22(4):373-7, 2009
                                                          8. Rahman AM et al: Craniofacial fibrous dysplasia: clinical characteristics and long-term outcomes. Eye (Lond). 23(12):2175-81, 2009
                                                          9. 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
                                                          10. 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
                                                          11. 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
                                                          12. Chapurlat RD et al: Fibrous dysplasia of bone and McCune-Albright syndrome. Best Pract Res Clin Rheumatol. 22(1):55-69, 2008
                                                          13. Daneman A et al: McCune-Albright syndrome. J Pediatr Endocrinol Metab. 20(12):1265, 2007
                                                          14. Defilippi C et al: Image diagnosis in McCune-Albright syndrome. J Pediatr Endocrinol Metab. 19 Suppl 2:561-70, 2006
                                                          15. 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
                                                          16. Kaushik S et al: Malignant transformation of fibrous dysplasia into chondroblastic osteosarcoma. Skeletal Radiol. 31(2):103-6, 2002
                                                          17. Sener RN: An extensive type of polyostotic fibrous dysplasia. Pediatr Radiol. 27(4):339-41, 1997
                                                          18. Sisayan R et al: Polyostotic fibrous dysplasia in McCune-Albright syndrome diagnosed by bone scintigraphy. Clin Nucl Med. 22(6):410-2, 1997
                                                          19. Carod J et al: [Neuroimaging, CT and MR brain findings in a case of McCune-Albright syndrome.] Rev Neurol. 24(132):979-81, 1996
                                                          20. 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