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Fibrous Dysplasia
Miral D. Jhaveri, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Fibrous dysplasia (FD)
            • Synonyms

              • Craniofacial fibrous dysplasia (CFD), osteitis fibrosa, osteodystrophy fibrosa
              • McCune-Albright syndrome (MAS): One of the most common FD syndromes
              • Jaffe-Lichtenstein dysplasia (monostotic FD)
            • Definitions

              • Congenital disorder characterized by expanding lesions with mixture of fibrous tissue and woven bone
              • Defect in osteoblastic differentiation and maturation
              • One of the most common fibroosseous lesions

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Thomsen MD et al: Clinical and radiological observations in a case series of 26 patients with fibrous dysplasia. Calcif Tissue Int. 94(4):384-95, 2014
                                              2. Fattah A et al: Paediatric craniofacial fibrous dysplasia: the Hospital for Sick Children experience and treatment philosophy. J Plast Reconstr Aesthet Surg. 66(10):1346-55, 2013
                                              3. Hanifi B et al: Craniofacial fibrous dysplasia. Clin Imaging. 37(6):1109-15, 2013
                                              4. Jhamb A et al: Craniofacial fibrous dysplasia. J Oral Maxillofac Pathol. 16(3):465-9, 2012
                                              5. Pruksakorn P et al: Craniofacial fibrous dysplasia. Intern Med. 49(3):249-50, 2010
                                              6. Dumitrescu CE et al: McCune-Albright syndrome. Orphanet J Rare Dis. 3:12, 2008
                                              7. Sargin H et al: A case of McCune-Albright syndrome associated with Gs alpha mutation in the bone tissue. Endocr J. 53(1):35-44, 2006
                                              8. DiCaprio MR et al: Fibrous dysplasia. Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am. 87(8):1848-64, 2005
                                              9. Karlberg N et al: Mulibrey nanism: clinical features and diagnostic criteria. J Med Genet. 41(2):92-8, 2004
                                              10. Kos M et al: Treatment of monostotic fibrous dysplasia with pamidronate. J Craniomaxillofac Surg. 32(1):10-5, 2004
                                              11. MacDonald-Jankowski DS: Fibro-osseous lesions of the face and jaws. Clin Radiol. 59(1):11-25, 2004
                                              12. Parekh SG et al: Fibrous dysplasia. J Am Acad Orthop Surg. 12(5):305-13, 2004
                                              13. Chattopadhyay A et al: Hypophosphatemic rickets and osteomalacia in polyostotic fibrous dysplasia. J Pediatr Endocrinol Metab. 16(6):893-6, 2003
                                              14. Riminucci M et al: FGF-23 in fibrous dysplasia of bone and its relationship to renal phosphate wasting. J Clin Invest. 112(5):683-92, 2003
                                              15. Lustig LR et al: Fibrous dysplasia involving the skull base and temporal bone. Arch Otolaryngol Head Neck Surg. 127(10):1239-47, 2001
                                              16. Bianco P et al: Mutations of the GNAS1 gene, stromal cell dysfunction, and osteomalacic changes in non-McCune-Albright fibrous dysplasia of bone. J Bone Miner Res. 15(1):120-8, 2000
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Fibrous dysplasia (FD)
                                                        • Synonyms

                                                          • Craniofacial fibrous dysplasia (CFD), osteitis fibrosa, osteodystrophy fibrosa
                                                          • McCune-Albright syndrome (MAS): One of the most common FD syndromes
                                                          • Jaffe-Lichtenstein dysplasia (monostotic FD)
                                                        • Definitions

                                                          • Congenital disorder characterized by expanding lesions with mixture of fibrous tissue and woven bone
                                                          • Defect in osteoblastic differentiation and maturation
                                                          • One of the most common fibroosseous lesions

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • MR Findings

                                                                • Nuclear Medicine Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Thomsen MD et al: Clinical and radiological observations in a case series of 26 patients with fibrous dysplasia. Calcif Tissue Int. 94(4):384-95, 2014
                                                                                          2. Fattah A et al: Paediatric craniofacial fibrous dysplasia: the Hospital for Sick Children experience and treatment philosophy. J Plast Reconstr Aesthet Surg. 66(10):1346-55, 2013
                                                                                          3. Hanifi B et al: Craniofacial fibrous dysplasia. Clin Imaging. 37(6):1109-15, 2013
                                                                                          4. Jhamb A et al: Craniofacial fibrous dysplasia. J Oral Maxillofac Pathol. 16(3):465-9, 2012
                                                                                          5. Pruksakorn P et al: Craniofacial fibrous dysplasia. Intern Med. 49(3):249-50, 2010
                                                                                          6. Dumitrescu CE et al: McCune-Albright syndrome. Orphanet J Rare Dis. 3:12, 2008
                                                                                          7. Sargin H et al: A case of McCune-Albright syndrome associated with Gs alpha mutation in the bone tissue. Endocr J. 53(1):35-44, 2006
                                                                                          8. DiCaprio MR et al: Fibrous dysplasia. Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am. 87(8):1848-64, 2005
                                                                                          9. Karlberg N et al: Mulibrey nanism: clinical features and diagnostic criteria. J Med Genet. 41(2):92-8, 2004
                                                                                          10. Kos M et al: Treatment of monostotic fibrous dysplasia with pamidronate. J Craniomaxillofac Surg. 32(1):10-5, 2004
                                                                                          11. MacDonald-Jankowski DS: Fibro-osseous lesions of the face and jaws. Clin Radiol. 59(1):11-25, 2004
                                                                                          12. Parekh SG et al: Fibrous dysplasia. J Am Acad Orthop Surg. 12(5):305-13, 2004
                                                                                          13. Chattopadhyay A et al: Hypophosphatemic rickets and osteomalacia in polyostotic fibrous dysplasia. J Pediatr Endocrinol Metab. 16(6):893-6, 2003
                                                                                          14. Riminucci M et al: FGF-23 in fibrous dysplasia of bone and its relationship to renal phosphate wasting. J Clin Invest. 112(5):683-92, 2003
                                                                                          15. Lustig LR et al: Fibrous dysplasia involving the skull base and temporal bone. Arch Otolaryngol Head Neck Surg. 127(10):1239-47, 2001
                                                                                          16. Bianco P et al: Mutations of the GNAS1 gene, stromal cell dysfunction, and osteomalacic changes in non-McCune-Albright fibrous dysplasia of bone. J Bone Miner Res. 15(1):120-8, 2000