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Neoplastic transformation and tumour-like lesions in Paget's disease of bone: a pictorial review

  • Musculoskeletal
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Abstract

The development of a sarcoma is the most serious complication of Paget's disease of bone. Although its incidence is <1% of those with the underlying disease, it is important to recognise the imaging features of these tumours as Paget's disease of bone is relatively common in the ageing population in certain parts of the world. The purpose of this pictorial review is to present the imaging features of Paget's sarcoma based on one orthopaedic oncology centres experience in 49 patients; however, not all masses or destructive lesions arising in association with Paget's disease are sarcomas and not all the tumours are malignant. This review also includes other malignancies which may arise in pagetic bone as well as tumour-like manifestations of Paget's disease.

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References

  1. Hamdy RC (1981) Paget's disease of bone: assessment and management. Praeger, UK, pp 1–2

  2. Paget J (1877) On a form of chronic inflammation of bones (osteitis deformans). Trans R Med Chir Soc 60:37–63

    Google Scholar 

  3. Paget J (1889) Remarks on osteitis deformans. Illust Med News 2:181–185

    Google Scholar 

  4. Wick MR, Siegal GP, Unni KK, McLeod RA, Greditzer G (1981) Sarcomas of bone complicating osteitis deformans (Paget's disease): 50 years experience. Am J Surg Pathol 5:47–57

    CAS  PubMed  Google Scholar 

  5. Greditzer HG, McLeod RA, Unni KK, Beabout JW (1983) Bone sarcomas in Paget's disease. Radiology 146:327–333

    PubMed  Google Scholar 

  6. Schajowicz F, Santini AE, Berenstein M (1983) Sarcoma complicating Paget's disease of bone: a clinicopathological study of 62 cases. J Bone Joint Surg 65B:299–306

    CAS  Google Scholar 

  7. Hadjipavlou A, Lander P, Srolovitz H, Enker P (1992) Malignant transformation in Paget's disease of bone. Cancer 70:802–808

    Google Scholar 

  8. Huvos AG, Butler A, Bretsky SS (1983) Osteogenic sarcoma associated with Paget's disease of bone: a clinicopathologic study of 65 cases. Cancer 52:1489–1495

    CAS  PubMed  Google Scholar 

  9. Haibach H, Farrell C, Dittrich FJ (1985) Neoplasm arising in Paget's disease of bone: a study of 82 cases. Am J Clin Pathol 83:594–600

    CAS  PubMed  Google Scholar 

  10. Hansen MF, Nellissery MJ, Bhatia P (1999) Common mechanisms of osteosarcoma and Paget's disease. J Bone Miner Res 14:39–44

    Google Scholar 

  11. Damron TA, McNairn J, Landas S, Shrimpton A (2001) Inheritance of Paget's osteosarcoma of bone: a familial linkage study. Proc 11th International Symposium on Limb Salvage, Birmingham, UK, pp 66–67

  12. Porretta CA, Dahlin DC, James JM (1957) Sarcoma in Paget's disease of bone. J Bone Joint Surg 39A:1314–1329

    Google Scholar 

  13. McKenna RJ, Schwinn CP, Soongy KY, Higinbotham NL (1964) Osteogenic sarcoma arising in Paget's disease. Cancer 17:42–66

    CAS  Google Scholar 

  14. Price CHG, Goldie W (1969) Paget's sarcoma of bone: a study of eighty cases from the Bristol and Leeds bone tumour register. J Bone Joint Surg 51B:205–224

    CAS  Google Scholar 

  15. Smith J, Botet JF, Yeh DJ (1984) Bone sarcomas in Paget's disease: a study of 85 patients. Radiology 152:583–590

    CAS  PubMed  Google Scholar 

  16. Jattiot F, Goupille P, Azais I, Roulot B, Alcalay M, Jeannou J, Bontoux D, Valat J (1999) Fourteen cases of sarcomatous degeneration in Paget's disease. J Rheumatol 26:150–155

    CAS  PubMed  Google Scholar 

  17. Choquette D, Haraoui B, Altman RD, Pelletier JP (1983) Multifocal sarcoma in Paget's disease: a hypothesis. Clin Orthop 179:308–311

    PubMed  Google Scholar 

  18. Moore TE, King AR, Kathol MH, El-Khoury GY, Palmer R, Downey PR (1991) Sarcoma in Paget's disease of bone: clinical radiologic and pathologic features in 22 cases. AJR 156:1199–1203

    CAS  Google Scholar 

  19. Erlich RB, Romano S, Meohas W, Smith J (1999) Synchronous Paget's sarcoma of tibiae in which Paget's disease was limited to these bones. Skeletal Radiol 28:599–603

    Article  CAS  PubMed  Google Scholar 

  20. Vuillemin-Bodaghi V, Parlier-Cuau C, Cywiner-Golenzer C, Quillard A, Kaplan G, Laredo JD (2000) Multifocal osteogenic sarcoma in Paget's disease. Skeletal Radiol 29:349–353

    Article  CAS  PubMed  Google Scholar 

  21. Huvos AG (1986) Osteogenic sarcoma of bones and soft tissues in older persons. A clinicopathologic analysis of 117 patients older than 60 years. Cancer 57:1442–1449

    CAS  PubMed  Google Scholar 

  22. Mirra JM, Brien EW, Tehranzadeh J (1995) Paget's disease of bone: review with emphasis on radiologic features, part II. Skeletal Radiol 24:173–184

    CAS  PubMed  Google Scholar 

  23. Yeh DJ, Rosen G, Benua RS (1982) Gallium scans in Paget's sarcoma. Clin Nucl Med 7:546–552

    CAS  PubMed  Google Scholar 

  24. Colarinha P, Fonseca AT, Salgado L, Vieira MR (1996) Diagnosis of malignant change in Paget's disease by Tl-201. Clin Nucl Med 21:299–301

    Article  CAS  PubMed  Google Scholar 

  25. Kaufmann GA, Sundaram M, McDonald DJ (1991) MR imaging in symptomatic Paget's disease. Skeletal Radiol 20:413–418

    CAS  PubMed  Google Scholar 

  26. Sundaram M, Khanna G, El-Khoury GY (2001) T1-weighted MR imaging for distinguishing large osteolysis of Paget's disease from sarcomatous degeneration. Skeletal Radiol 30:378–383

    Article  CAS  PubMed  Google Scholar 

  27. Van de Berg BC, Malghem J, Lecouvet FE, Maldague B (2001) MR appearance of uncomplicated Paget's disease of Bone. Semin Musculoskel Radiol 5:69–77

    Article  Google Scholar 

  28. Boutin RD, Spitz DJ, Newman JS, Lenchik L, Steinbach LS (1998) Complications in Paget's disease at MR imaging. Radiology 209:641–651

    CAS  PubMed  Google Scholar 

  29. Shaylor PJ, Peake D, Grimer RJ, Carter SR, Tillman RM, Spooner D (1999) Paget's osteosarcoma: no cure in sight. Sarcoma 3:191–192

    Article  Google Scholar 

  30. Burgener FA, Perry PE (1977) Solitary renal metastasis in Paget's disease simulating sarcomatous degeneration. AJR 128:853–855

    CAS  Google Scholar 

  31. Nicholas JJ, Srodes CH, Herbert D, Hoy RJ, Peel RL, Goodman MA (1987) Metastatic cancer in Paget's disease of bone: a case report. Orthopedics 10:725–729

    CAS  PubMed  Google Scholar 

  32. Roblot P, Alcay M, Payen J, Clarac JP, Fontanel JP, Bontoux D (1987) Metastases of a lingual epidermoid carcinoma in a femur affected by Paget's disease: report of a case. J Bone Joint Surg 69A:1440–1441

    CAS  PubMed  Google Scholar 

  33. Fenton P, Resnick D (1991) Metastases to bone affected by Paget's disease: a report of three cases. Int Orthop 15:397–399

    CAS  PubMed  Google Scholar 

  34. Conrad GR, Johnson AW (1997) Solitary adenocarcinoma metastasis mimicking sarcomatous degeneration in Paget's disease. Clin Nucl Med 22:300–302

    Article  CAS  PubMed  Google Scholar 

  35. Schajowicz F, Velan O, Santini AE et al. (1988) Metastases of carcinoma in the pagetic bone: a report of two cases. Clin Orthop 228:290–296

    PubMed  Google Scholar 

  36. Jacobson HG, Siegelmann SS (1966) Some miscellaneous solitary bone lesions. Semin Roentgenol 1:314–335

    Google Scholar 

  37. Moore TE, Kathol MH, El-Khoury GY, Walker CW, Gendall PW, Whitten CG (1994) Unusual radiological features in Paget's disease of bone. Skeletal Radiol 23:257–260

    CAS  PubMed  Google Scholar 

  38. Singer FR (1977) Paget's disease of bone. Plenum, New York, pp 99–102

  39. Rosenkrantz JA, Gluckman EC (1957) Co-existence of Paget's disease of bone and multiple myeloma. AJR 78:30–38

    Google Scholar 

  40. Cazeilles M, Cornet L, Javel R, Pene J (1953) Leucémie lymphoide et ostéopathia à type de maladie de Paget avec ostéoporose circonstrite du crane de Schüller. J Med Bordeaux 130:1012–1015

    Google Scholar 

  41. Stephens GC, Lennington WJ, Schwartz HS (1994) Primary lymphoma and Paget's disease of the femur. Am J Clin Pathol 101:783–786

    CAS  PubMed  Google Scholar 

  42. Lauchlan SC, Walsh MJ (1963) Reticulum cell sarcoma complicating Paget's disease. Can Med Assoc 88:891–892

    Google Scholar 

  43. Molle D, Bard H, Kuntz D et al. (1983) Osteolyse relatrice d'un lymphome developpe sur un os pagetique. Rev Rhum Mal Osteoartic 50:217–221

    CAS  PubMed  Google Scholar 

  44. Gebhart M, Vandeweyer E, Nemec E (1998) Paget's disease of bone complicated by giant cell tumor. Clin Orthop 352:187–193

    PubMed  Google Scholar 

  45. Dixon GR, Ritchie DA, Myskow MW (1995) Case report: benign giant cell tumour associated with Paget's disease of bone. Clin Radiol 50:269–271

    CAS  PubMed  Google Scholar 

  46. Ueda T, Healey JH, Huvos AG, Panicek DM (1996) Orthopaedic, radiology, pathology conference: scapular pain and swelling in a 60-year-old man with Paget's disease. Clin Orthop 326:284–286, 310–312

    PubMed  Google Scholar 

  47. Jacobs TP, Michelson J, Polay JS, d'Adamo AC, Canfield RE (1979) Giant cell tumor in Paget's disease of bone: familial and geographical clustering. Cancer 44:742–747

    CAS  PubMed  Google Scholar 

  48. Potter HG, Schneider R, Ghelman B, Healey JH, Lane JM (1991) Multiple giant cell tumors and Paget's disease of bone: radiographic and clinical correlations. Radiology 180:261–264

    CAS  PubMed  Google Scholar 

  49. Chiari A de, Apice U, Fazioli F et al. (1998) Multicentric giant cell tumour with viral-like inclusions associated with Paget's disease of bone: a case treated with steroid therapy. Oncol Rep 5:317–320

    PubMed  Google Scholar 

  50. Zehr RJ, Bauer TW, Recht MP (2000) Enchondroma associated with Paget's disease. Orthopedics 23:1287–1290

    CAS  PubMed  Google Scholar 

  51. Mirra JM, Finerman G, Lindholm S (1980) Diffuse pigmented villonodular synovitis in association with Paget's disease of bone: a report of a case. Clin Orthop 149:305–309

    PubMed  Google Scholar 

  52. Hillmann JS, Mesgarzadeh M, Tang CK, Bonakdapour A, Reyes TG (1988) Case report 481: benign intraosseous fibroma (desmoplastic fibroma) associated with Paget's disease of the iliac bone. Skeletal Radiol 17:356–359

    CAS  PubMed  Google Scholar 

  53. Marsal S, Arnal C, Garcia F, Azorin L, Lience E (1993) Pigmented villonodular synovitis of the knee in a patient with Paget's disease of the adjacent femoral bone. Br J Rheumatol 32:166–168

    CAS  PubMed  Google Scholar 

  54. Mirra JM, Brien EW, Tehranzadeh J (1995) Paget's disease of bone: review with emphasis on radiologic features, part I. Skeletal Radiol 24:163–171

    CAS  PubMed  Google Scholar 

  55. Bowerman JW, Altman J, Hughes JL, Zadek RE (1975) Pseudomalignant lesions in Paget's disease of bone. AJR 124:57–61

    CAS  Google Scholar 

  56. Marin F, Billey T, Lassoued S (1996) Ostéolyse pseudosarcomateuse du femur chez un pagétique traité au long cours par étidronate disodique: à propos d'une nouvelle observation. Rev Méd Interne 17:856–858

  57. Eisman JA, Martin T (1986) Osteolytic Paget's disease. J Bone Joint Surg 68B:112–117

    CAS  PubMed  Google Scholar 

  58. Kumar A, Poon PY, Aggarwal S (1993) Value of CT in diagnosing nonneoplastic osteolysis in Paget's disease. J Comput Assist Tomogr 17:144–146

    CAS  PubMed  Google Scholar 

  59. Boutry N, Moisan-Lemanissier S, Leroy X, Paul C, Lecomte-Houcke M, Cotton A (2000) "Inflammatory changes" in Paget's disease of bone: a frequent MR finding on fat suppression techniques. Radiology 217:269–270

    Google Scholar 

  60. Tins BJ, Davies AM, Mangham DC (2001) MR imaging of pseudosarcoma in Paget's disease of bone: a report of 2 cases. Skeletal Radiol 30:161–165

    Article  CAS  PubMed  Google Scholar 

  61. Golding C (1960) Museum pages: IV. On the differential diagnosis of Paget's disease. J Bone Joint Surg 42B:641–643

    Google Scholar 

  62. Milgram JW (1977) Radiographical and pathological assessment of the activity of Paget's disease of bone. Clin Orthop 127:43–54

    PubMed  Google Scholar 

  63. Resnick CS, Walter RD, Haghighi P, Resnick D (1982) Paget's disease with soft tissue extension: case report 218. Skeletal Radiol 9:145–147

    CAS  PubMed  Google Scholar 

  64. Monson DK, Finn HA, Dawson PJ, Simon MA (1989) Pseudosarcoma in Paget's disease of bone. J Bone Joint Surg 71A:453–455

    CAS  PubMed  Google Scholar 

  65. Khraishi M, Howard B, Fam AG (1991) Paget's pseudosarcoma. Arthritis Rheum 34:241–243

    CAS  PubMed  Google Scholar 

  66. Resnick D (1995) Diagnosis of bone and joint disorders, 3rd edn. Saunders, Philadelphia, p 1953

  67. Lamovec J, Renner M, Spiler M (1999) Pseudosarcoma in Paget's disease of bone. Ann Diagn Pathol 3:99–103

    CAS  PubMed  Google Scholar 

  68. Donáth J, Szilágyi M, Fornet B, Bély M, Poór G (2000) Pseudosarcoma in Paget's disease: case report. Eur Radiol 10:1664–1668

    Article  PubMed  Google Scholar 

  69. McNairn JDK, Damron TA, Landa SK, Ambrose JL (2001) Benign tumefactive soft tissue extension from Paget's disease of bone simulating malignancy. Skeletal Radiol 30:157–160

    Article  CAS  PubMed  Google Scholar 

  70. Sear HR (1949) Osteogenic sarcoma as a complication of osteitis deformans: some personal experiences and reflections. Br J Radiol 22:580–587

    Google Scholar 

  71. Mitchell ML, Ackerman LV,Tsutsumi A (1987) Case report 438: osteolytic phase of Paget's disease. Skeletal Radiol 16:498–503

    CAS  PubMed  Google Scholar 

  72. Wallace K, Haddad JG, Gannon FH, Esterhai J, Kaplan FS (1996) Skeletal response to immobilization in Paget's disease of bone: a case report. Clin Orthop 328:236–240

    PubMed  Google Scholar 

  73. Smith R, Russell RGG, Bishop M (1971) Diphosphonates and Paget's disease of bone. Lancet 1:945–947

    CAS  Google Scholar 

  74. Hadjipavlou AG, Gaitanis IN, Kontakis GM (2002) Review article: Paget's disease of bone and its management. J Bone Joint Surg 84B:160–169

    Article  Google Scholar 

  75. MacGowan JR, Pringle J, Morris VH, Stamp TCB (2000) Gross vertebral collapse associated with long-term disodium etidronate treatment for pelvic Paget's disease. Skeletal Radiol 29:279–282

    Article  CAS  PubMed  Google Scholar 

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  1. Department of Radiology, Royal Orthopaedic Hospital, Birmingham, B31 2AP, UK

    C. López, D. V. Thomas & A. M. Davies

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  1. C. López
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  2. D. V. Thomas
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  3. A. M. Davies
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Correspondence to A. M. Davies.

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López, C., Thomas, D.V. & Davies, A.M. Neoplastic transformation and tumour-like lesions in Paget's disease of bone: a pictorial review. Eur Radiol 13 (Suppl 6), L151–L163 (2003). https://doi.org/10.1007/s00330-003-1927-3

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  • DOI: https://doi.org/10.1007/s00330-003-1927-3

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