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Head and Neck
Diagnosis
Skull Base Lesions
Diffuse or Multifocal Skull Base Disease
Skull Base Fibrous Dysplasia
Skull Base Fibrous Dysplasia
C. Douglas Phillips, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Fibrous dysplasia (FD)

            • Synonyms

              • Craniofacial FD (CFD), osteitis fibrosa, osteodystrophy fibrosa; McCune-Albright syndrome
              • Jaffe-Lichtenstein dysplasia

            • Definitions

              • Congenital disorder with defect in osteoblastic differentiation and maturation, resulting in progressive replacement of normal cancellous bone by mixture of fibrous tissue and immature woven bone

            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

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Chapurlat R et al: Bisphosphonates for the treatment of fibrous dysplasia of bone. 143:115784, 2021
                                            2. Chattopadhyay A et al: Craniofacial fibrous dysplasia. J Clin Rheumatol. 26(7):e214, 2020
                                            3. Davidova LA et al: An analysis of clinical and histopathologic features of fibrous dysplasia of the jaws: a series of 40 cases and review of literature. Head Neck Pathol. 14(2):353-61, 2020
                                            4. Hwang D et al: Radiographic follow-up of fibrous dysplasia in 138 patients. AJR Am J Roentgenol. 215(6):1430-5, 2020
                                            5. Lee SE et al: The diagnostic utility of the GNAS mutation in patients with fibrous dysplasia: meta-analysis of 168 sporadic cases. Hum Pathol. 43(8):1234-42, 2012
                                            6. Wei YT et al: Fibrous dysplasia of skull. J Craniofac Surg. 21(2):538-42, 2010
                                            7. Lisle DA et al: Imaging of craniofacial fibrous dysplasia. J Med Imaging Radiat Oncol. 52(4):325-32, 2008
                                            8. Hullar TE et al: Paget's disease and fibrous dysplasia. Otolaryngol Clin North Am. 36(4):707-32, 2003
                                            9. Chong VF et al: Fibrous dysplasia involving the base of the skull. AJR Am J Roentgenol. 178(3):717-20, 2002
                                            10. Itshayek E et al: Fibrous dysplasia in combination with aneurysmal bone cyst of the occipital bone and the clivus: case report and review of the literature. Neurosurgery. 51(3):815-7; discussion 817-8, 2002
                                            11. Sharma RR et al: Symptomatic cranial fibrous dysplasias: clinico-radiological analysis in a series of eight operative cases with follow-up results. J Clin Neurosci. 9(4):381-90, 2002
                                            12. Sirvanci M et al: Monostotic fibrous dysplasia of the clivus: MRI and CT findings. Neuroradiology. 44(10):847-50, 2002
                                            13. Tsuyuguchi N et al: Magnetic resonance imaging and [11C]methyl-L-methionine positron emission tomography of fibrous dysplasia--two case reports. Neurol Med Chir (Tokyo). 42(8):341-5, 2002
                                            14. Lustig LR et al: Fibrous dysplasia involving the skull base and temporal bone. Arch Otolaryngol Head Neck Surg. 127(10):1239-47, 2001
                                            15. Haddad GF et al: Concomitant fibrous dysplasia and aneurysmal bone cyst of the skull base. Case report and review of the literature. Pediatr Neurosurg. 28(3):147-53, 1998
                                            16. Ham DW et al: Fibrous dysplasia of the clivus and sphenoid sinus. Mil Med. 163(3):186-9, 1998
                                            17. Jee WH et al: Fibrous dysplasia: MR imaging characteristics with radiopathologic correlation. AJR Am J Roentgenol. 167(6):1523-7, 1996
                                            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 FD (CFD), osteitis fibrosa, osteodystrophy fibrosa; McCune-Albright syndrome
                                                        • Jaffe-Lichtenstein dysplasia

                                                      • Definitions

                                                        • Congenital disorder with defect in osteoblastic differentiation and maturation, resulting in progressive replacement of normal cancellous bone by mixture of fibrous tissue and immature woven bone

                                                      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

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Chapurlat R et al: Bisphosphonates for the treatment of fibrous dysplasia of bone. 143:115784, 2021
                                                                                      2. Chattopadhyay A et al: Craniofacial fibrous dysplasia. J Clin Rheumatol. 26(7):e214, 2020
                                                                                      3. Davidova LA et al: An analysis of clinical and histopathologic features of fibrous dysplasia of the jaws: a series of 40 cases and review of literature. Head Neck Pathol. 14(2):353-61, 2020
                                                                                      4. Hwang D et al: Radiographic follow-up of fibrous dysplasia in 138 patients. AJR Am J Roentgenol. 215(6):1430-5, 2020
                                                                                      5. Lee SE et al: The diagnostic utility of the GNAS mutation in patients with fibrous dysplasia: meta-analysis of 168 sporadic cases. Hum Pathol. 43(8):1234-42, 2012
                                                                                      6. Wei YT et al: Fibrous dysplasia of skull. J Craniofac Surg. 21(2):538-42, 2010
                                                                                      7. Lisle DA et al: Imaging of craniofacial fibrous dysplasia. J Med Imaging Radiat Oncol. 52(4):325-32, 2008
                                                                                      8. Hullar TE et al: Paget's disease and fibrous dysplasia. Otolaryngol Clin North Am. 36(4):707-32, 2003
                                                                                      9. Chong VF et al: Fibrous dysplasia involving the base of the skull. AJR Am J Roentgenol. 178(3):717-20, 2002
                                                                                      10. Itshayek E et al: Fibrous dysplasia in combination with aneurysmal bone cyst of the occipital bone and the clivus: case report and review of the literature. Neurosurgery. 51(3):815-7; discussion 817-8, 2002
                                                                                      11. Sharma RR et al: Symptomatic cranial fibrous dysplasias: clinico-radiological analysis in a series of eight operative cases with follow-up results. J Clin Neurosci. 9(4):381-90, 2002
                                                                                      12. Sirvanci M et al: Monostotic fibrous dysplasia of the clivus: MRI and CT findings. Neuroradiology. 44(10):847-50, 2002
                                                                                      13. Tsuyuguchi N et al: Magnetic resonance imaging and [11C]methyl-L-methionine positron emission tomography of fibrous dysplasia--two case reports. Neurol Med Chir (Tokyo). 42(8):341-5, 2002
                                                                                      14. Lustig LR et al: Fibrous dysplasia involving the skull base and temporal bone. Arch Otolaryngol Head Neck Surg. 127(10):1239-47, 2001
                                                                                      15. Haddad GF et al: Concomitant fibrous dysplasia and aneurysmal bone cyst of the skull base. Case report and review of the literature. Pediatr Neurosurg. 28(3):147-53, 1998
                                                                                      16. Ham DW et al: Fibrous dysplasia of the clivus and sphenoid sinus. Mil Med. 163(3):186-9, 1998
                                                                                      17. Jee WH et al: Fibrous dysplasia: MR imaging characteristics with radiopathologic correlation. AJR Am J Roentgenol. 167(6):1523-7, 1996