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Paget Disease
Behrang Amini, MD, PhD; B. J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Paget disease of bone (PDB), osteitis deformans
          • Definitions

            • Focal disorder of bone metabolism characterized by accelerated remodeling, resulting in overgrowth
          • Associated Syndromes

            • Multisystem proteinopathy: rare disease, variably including PDB, inclusion body myopathy, motor neuron disease, parkinsonism, and frontotemporal dementia

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Nuclear Medicine Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Image Interpretation Pearls

                                    Selected References

                                    1. Skaggs JW et al: Paget disease of bone: current concepts, CDR 42 (17): 1-7, 2019
                                    2. Rendina D et al: Clinical characteristics and evolution of giant cell tumor occurring in paget's disease of bone. J Bone Miner Res. 30(2):257-63, 2015
                                    3. Galson DL et al: Pathobiology of Paget's disease of bone. J Bone Metab. 21(2):85-98, 2014
                                    4. Bolland MJ et al: Paget's disease of bone: clinical review and update. J Clin Pathol. 66(11):924-7, 2013
                                    5. Goode A et al: Recent advances in understanding the molecular basis of Paget disease of bone. J Clin Pathol. 63(3):199-203, 2010
                                    6. Bastin S et al: Paget's disease of bone--becoming a rarity? Rheumatology (Oxford). 48(10):1232-5, 2009
                                    7. Singer FR: Paget disease: when to treat and when not to treat. Nat Rev Rheumatol. 5(9):483-9, 2009
                                    8. Libicher M et al: Dynamic contrast-enhanced MRI in Paget's disease of bone--correlation of regional microcirculation and bone turnover. Eur Radiol. 18(5):1005-11, 2008
                                    9. Haddaway MJ et al: Effect of age and gender on the number and distribution of sites in Paget's disease of bone. Br J Radiol. 80(955):532-6, 2007
                                    10. Forest M et al: Secondary osteosarcomas. In Fletcher CDM et al: World Health Organization Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 277, 2002
                                    11. Smith SE et al: From the archives of the AFIP. Radiologic spectrum of Paget disease of bone and its complications with pathologic correlation. Radiographics. 22(5):1191-216, 2002
                                    12. Whitehouse RW: Paget's disease of bone. Semin Musculoskelet Radiol. 6(4):313-22, 2002
                                    13. Sundaram M et al: T1-weighted MR imaging for distinguishing large osteolysis of Paget's disease from sarcomatous degeneration. Skeletal Radiol. 30(7):378-83, 2001
                                    14. Tins BJ et al: MR imaging of pseudosarcoma in Paget's disease of bone: a report of two cases. Skeletal Radiol. 30(3):161-5, 2001
                                    15. Vande Berg BC et al: Magnetic resonance appearance of uncomplicated Paget's disease of bone. Semin Musculoskelet Radiol. 5(1):69-77, 2001
                                    16. Donáth J et al: Pseudosarcoma in Paget's disease. Eur Radiol. 10(10):1664-8, 2000
                                    17. Boutin RD et al: Complications in Paget disease at MR imaging. Radiology. 209(3):641-51, 1998
                                    18. Mirra JM et al: Paget's disease of bone: review with emphasis on radiologic features, part I. Skeletal Radiol. 24(3):163-71, 1995
                                    19. Mirra JM et al: Paget's disease of bone: review with emphasis on radiologic features, part II. Skeletal Radiol. 24(3):173-84, 1995
                                    20. Kaufmann GA et al: Magnetic resonance imaging in symptomatic Paget's disease. Skeletal Radiol. 20(6):413-8, 1991
                                    21. Moore TE et al: Sarcoma in Paget disease of bone: clinical, radiologic, and pathologic features in 22 cases. AJR Am J Roentgenol. 156(6):1199-203, 1991
                                    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

                                            TERMINOLOGY

                                            • Synonyms

                                              • Paget disease of bone (PDB), osteitis deformans
                                            • Definitions

                                              • Focal disorder of bone metabolism characterized by accelerated remodeling, resulting in overgrowth
                                            • Associated Syndromes

                                              • Multisystem proteinopathy: rare disease, variably including PDB, inclusion body myopathy, motor neuron disease, parkinsonism, and frontotemporal dementia

                                            IMAGING

                                            • General Features

                                              • Radiographic Findings

                                                • CT Findings

                                                  • MR Findings

                                                    • Nuclear Medicine Findings

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Microscopic Features

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Image Interpretation Pearls

                                                                      Selected References

                                                                      1. Skaggs JW et al: Paget disease of bone: current concepts, CDR 42 (17): 1-7, 2019
                                                                      2. Rendina D et al: Clinical characteristics and evolution of giant cell tumor occurring in paget's disease of bone. J Bone Miner Res. 30(2):257-63, 2015
                                                                      3. Galson DL et al: Pathobiology of Paget's disease of bone. J Bone Metab. 21(2):85-98, 2014
                                                                      4. Bolland MJ et al: Paget's disease of bone: clinical review and update. J Clin Pathol. 66(11):924-7, 2013
                                                                      5. Goode A et al: Recent advances in understanding the molecular basis of Paget disease of bone. J Clin Pathol. 63(3):199-203, 2010
                                                                      6. Bastin S et al: Paget's disease of bone--becoming a rarity? Rheumatology (Oxford). 48(10):1232-5, 2009
                                                                      7. Singer FR: Paget disease: when to treat and when not to treat. Nat Rev Rheumatol. 5(9):483-9, 2009
                                                                      8. Libicher M et al: Dynamic contrast-enhanced MRI in Paget's disease of bone--correlation of regional microcirculation and bone turnover. Eur Radiol. 18(5):1005-11, 2008
                                                                      9. Haddaway MJ et al: Effect of age and gender on the number and distribution of sites in Paget's disease of bone. Br J Radiol. 80(955):532-6, 2007
                                                                      10. Forest M et al: Secondary osteosarcomas. In Fletcher CDM et al: World Health Organization Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 277, 2002
                                                                      11. Smith SE et al: From the archives of the AFIP. Radiologic spectrum of Paget disease of bone and its complications with pathologic correlation. Radiographics. 22(5):1191-216, 2002
                                                                      12. Whitehouse RW: Paget's disease of bone. Semin Musculoskelet Radiol. 6(4):313-22, 2002
                                                                      13. Sundaram M et al: T1-weighted MR imaging for distinguishing large osteolysis of Paget's disease from sarcomatous degeneration. Skeletal Radiol. 30(7):378-83, 2001
                                                                      14. Tins BJ et al: MR imaging of pseudosarcoma in Paget's disease of bone: a report of two cases. Skeletal Radiol. 30(3):161-5, 2001
                                                                      15. Vande Berg BC et al: Magnetic resonance appearance of uncomplicated Paget's disease of bone. Semin Musculoskelet Radiol. 5(1):69-77, 2001
                                                                      16. Donáth J et al: Pseudosarcoma in Paget's disease. Eur Radiol. 10(10):1664-8, 2000
                                                                      17. Boutin RD et al: Complications in Paget disease at MR imaging. Radiology. 209(3):641-51, 1998
                                                                      18. Mirra JM et al: Paget's disease of bone: review with emphasis on radiologic features, part I. Skeletal Radiol. 24(3):163-71, 1995
                                                                      19. Mirra JM et al: Paget's disease of bone: review with emphasis on radiologic features, part II. Skeletal Radiol. 24(3):173-84, 1995
                                                                      20. Kaufmann GA et al: Magnetic resonance imaging in symptomatic Paget's disease. Skeletal Radiol. 20(6):413-8, 1991
                                                                      21. Moore TE et al: Sarcoma in Paget disease of bone: clinical, radiologic, and pathologic features in 22 cases. AJR Am J Roentgenol. 156(6):1199-203, 1991