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Fibrous Dysplasia
Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            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. Cleven AHG et al: Molecular findings in maxillofacial bone tumours and its diagnostic value. Virchows Arch. 476(1):159-74, 2020
                                              2. Gutierrez LB et al: Secondary aneurysmal bone cysts and associated primary lesions: imaging features of 49 cases. Clin Imaging. 62:23-32, 2020
                                              3. Kinnunen AR et al: Magnetic resonance imaging characteristics in patients with histopathologically proven fibrous dysplasia-a systematic review. Skeletal Radiol. 49(6):837-45, 2020
                                              4. Pons Escoda A et al: Imaging of skull vault tumors in adults. Insights Imaging. 11(1):23, 2020
                                              5. van der Bruggen W et al: Quantifying skeletal burden in fibrous dysplasia using sodium fluoride PET/CT. Eur J Nucl Med Mol Imaging. 47(6):1527-37, 2020
                                              6. Butscheidt S et al: Primary intraosseous meningioma: clinical, histological, and differential diagnostic aspects. J Neurosurg. 1-10, 2019
                                              7. Franz D et al: Milk cloud appearance-a characteristic sign of fibrous dysplasia on contrast-enhanced MR imaging. Eur Radiol. 29(7):3424-30, 2019
                                              8. Gohil A et al: FGF23 and associated disorders of phosphate wasting. Pediatr Endocrinol Rev. 17(1):17-34, 2019
                                              9. 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
                                              10. Kabali TM et al: Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports. BMC Oral Health. 19(1):180, 2019
                                              11. Pereira TDSF et al: Fibrous dysplasia of the jaws: Integrating molecular pathogenesis with clinical, radiological, and histopathological features. J Oral Pathol Med. 48(1):3-9, 2019
                                              12. Kushchayeva YS et al: Fibrous dysplasia for radiologists: beyond ground glass bone matrix. Insights Imaging. 9(6):1035-56, 2018
                                              13. Ostertag H et al: [The activating GNAS mutation: a survey of fibrous dysplasia, its associated syndromes, and other skeletal and extraskeletal lesions.] Pathologe. 39(2):146-53, 2018
                                              14. Rowe SP et al: 3D cinematic rendering of the calvarium, maxillofacial structures, and skull base: preliminary observations. Br J Radiol. 91(1086):20170826, 2018
                                              15. Wilson M et al: Fibro-osseous lesions of the skull base in the pediatric population. J Neurol Surg B Skull Base. 79(1):31-6, 2018
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Clinical Issues

                                                        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. Cleven AHG et al: Molecular findings in maxillofacial bone tumours and its diagnostic value. Virchows Arch. 476(1):159-74, 2020
                                                                                          2. Gutierrez LB et al: Secondary aneurysmal bone cysts and associated primary lesions: imaging features of 49 cases. Clin Imaging. 62:23-32, 2020
                                                                                          3. Kinnunen AR et al: Magnetic resonance imaging characteristics in patients with histopathologically proven fibrous dysplasia-a systematic review. Skeletal Radiol. 49(6):837-45, 2020
                                                                                          4. Pons Escoda A et al: Imaging of skull vault tumors in adults. Insights Imaging. 11(1):23, 2020
                                                                                          5. van der Bruggen W et al: Quantifying skeletal burden in fibrous dysplasia using sodium fluoride PET/CT. Eur J Nucl Med Mol Imaging. 47(6):1527-37, 2020
                                                                                          6. Butscheidt S et al: Primary intraosseous meningioma: clinical, histological, and differential diagnostic aspects. J Neurosurg. 1-10, 2019
                                                                                          7. Franz D et al: Milk cloud appearance-a characteristic sign of fibrous dysplasia on contrast-enhanced MR imaging. Eur Radiol. 29(7):3424-30, 2019
                                                                                          8. Gohil A et al: FGF23 and associated disorders of phosphate wasting. Pediatr Endocrinol Rev. 17(1):17-34, 2019
                                                                                          9. 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
                                                                                          10. Kabali TM et al: Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports. BMC Oral Health. 19(1):180, 2019
                                                                                          11. Pereira TDSF et al: Fibrous dysplasia of the jaws: Integrating molecular pathogenesis with clinical, radiological, and histopathological features. J Oral Pathol Med. 48(1):3-9, 2019
                                                                                          12. Kushchayeva YS et al: Fibrous dysplasia for radiologists: beyond ground glass bone matrix. Insights Imaging. 9(6):1035-56, 2018
                                                                                          13. Ostertag H et al: [The activating GNAS mutation: a survey of fibrous dysplasia, its associated syndromes, and other skeletal and extraskeletal lesions.] Pathologe. 39(2):146-53, 2018
                                                                                          14. Rowe SP et al: 3D cinematic rendering of the calvarium, maxillofacial structures, and skull base: preliminary observations. Br J Radiol. 91(1086):20170826, 2018
                                                                                          15. Wilson M et al: Fibro-osseous lesions of the skull base in the pediatric population. J Neurol Surg B Skull Base. 79(1):31-6, 2018