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Osteofibrous Dysplasia Treatment & Management

  • Author: Darin Davidson, MD; Chief Editor: Harris Gellman, MD  more...
Updated: Mar 11, 2016

Medical Therapy

Nonoperative treatment usually is recommended until skeletal maturity is reached. Recurrent pathologic fractures may be an ongoing problem in some active children. Using a tibial brace similar to those used for congenital pseudarthrosis of the tibia may minimize recurrent pathologic fractures. A lace-up leather support from just below the knee to the ankle may be used. Fractures usually are nondisplaced and can be treated in a walking patellar tendon-bearing cast. Cast immobilization is sufficient for fracture healing, though healing is slower than normal.


Surgical Therapy

There are no absolute contraindications to surgical intervention in children, with the exception of any underlying medical or anesthetic issues. Operative management is not recommended in patients who are skeletally immature, because of the high recurrence rate after resection and curettage[56] and because of the predisposition to fracturing after the bone has been weakened by biopsy. Once skeletal maturity has been reached, marginal resection and bone grafting may be performed without increased risk of recurrence.[57, 58] Pathologic fracture does not necessarily require surgical management, because cast immobilization frequently results in good healing.

For patients of any age, surgical correction of associated deformities may be required. Campanacci and Laus recommended wide resection with extensive bone grafting in children who are skeletally immature if the lesion is aggressive, with marked expansion and bone destruction or multiple pathologic fractures.[4, 59] Intramedullary prophylactic rodding of the tibia may also be an option in children who frequently present with fractures; this approach is similar to that used in osteogenesis imperfecta. Resection of large portions of the lesion usually is not necessary and only increases susceptibility to recurrent fractures.



The recurrence rate after resection and curettage has been reported to be 64-100%. Goergen et al reported multiple recurrences in a 3-year-old boy and a 6-month-old boy after attempts at resection.[60] Wang et al also reported multiple recurrences after surgical intervention.[9] Campanacci and Laus indicated that recurrence does not develop in patients older than 10 years.[4]

Malignant transformation of the lesion is very rare. Ben Arush et al described the course of a boy diagnosed at age 4 years with osteofibrous dysplasia of the tibia who subsequently presented at age 14 years with synovial sarcoma of the peroneal muscles of the same leg.[61] At the time of the latter diagnosis, computed tomography (CT) confirmed multiple pulmonary metastases. Malignant transformation to soft-tissue sarcoma has been reported in fibrous dysplasia, most commonly in the polyostotic variation.[62, 63, 64] However, the case reported by Ben Arush et al is the only report of sarcomatous degeneration of osteofibrous dysplasia.

Contributor Information and Disclosures

Darin Davidson, MD Resident Physician, Department of Orthopedics, University of British Columbia Faculty of Medicine, Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Sean P Scully, MD 

Sean P Scully, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, International Society on Thrombosis and Haemostasis, Society of Surgical Oncology

Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami, Leonard M Miller School of Medicine, Clinical Professor, Surgery, Nova Southeastern School of Medicine

Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, Arkansas Medical Society

Disclosure: Nothing to disclose.


Robert Mervyn Letts, MD, FRCS(C), FACS Former Chief, Department of Surgery, Division of Pediatric Orthopedics, Children's Hospital of Eastern Ontario, University of Ottawa; Consultant Pediatric Orthopedic Surgeon, Sheikh Khalifa Medical City, UAE

Disclosure: Nothing to disclose.

  1. McCaffrey M, Letts M, Carpenter B, et al. Osteofibrous dysplasia: a review of the literature and presentation of an additional 3 cases. Am J Orthop. 2003 Oct. 32(10):479-86. [Medline].

  2. Frangenheim P. Angeborene Ostitis Fibrosa als Ursache einer Intrauterinen Unterschenkelfraktur. Arch Klin Chir. 1921. 117:22-9.

  3. Kempson RL. Ossifying fibroma of the long bones. A light and electron microscopic study. Arch Pathol. 1966 Sep. 82(3):218-33. [Medline].

  4. Campanacci M, Laus M. Osteofibrous dysplasia of the tibia and fibula. J Bone Joint Surg [Am]. 1981 Mar. 63(3):367-75. [Medline].

  5. Campanacci M. Osteofibrous dysplasia of long bones a new clinical entity. Ital J Orthop Traumatol. 1976 Aug. 2(2):221-37. [Medline].

  6. Sweet DE, Vinh TN, Devaney K. Cortical osteofibrous dysplasia of long bone and its relationship to adamantinoma. A clinicopathologic study of 30 cases. Am J Surg Pathol. 1992 Mar. 16(3):282-90. [Medline].

  7. Ishida T, Iijima T, Kikuchi F. A clinicopathological and immunohistochemical study of osteofibrous dysplasia, differentiated adamantinoma, and adamantinoma of long bones. Skeletal Radiol. 1992. 21(8):493-502. [Medline].

  8. Ozaki T, Hamada M, Sugihara S. Treatment outcome of osteofibrous dysplasia. J Pediatr Orthop B. 1998 Jul. 7(3):199-202. [Medline].

  9. Wang JW, Shih CH, Chen WJ. Osteofibrous dysplasia (ossifying fibroma of long bones). A report of four cases and review of the literature. Clin Orthop. 1992 May. (278):235-43. [Medline].

  10. Schlitter HE. [2 Benign bone tumors with unusual localization & spontaneous fracture.]. Fortschr Geb Rontgenstr Nuklearmed. 1958 Feb. 88(2):195-200. [Medline].

  11. Hunter AG, Jarvis J. Osteofibrous dysplasia: two affected male sibs and an unrelated girl with bilateral involvement. Am J Med Genet. 2002 Sep 15. 112(1):79-85. [Medline].

  12. Karol LA, Brown DS, Wise CA, Waldron M. Familial osteofibrous dysplasia. A case series. J Bone Joint Surg Am. 2005 Oct. 87 (10):2297-307. [Medline].

  13. Johnson LC. Congenital pseudarthrosis, adamantinoma of long bone and intracortical fibrous dysplasia of the tibia. J Bone Joint Surg. 1972. 54-A:1355.

  14. Komiya S, Inoue A. Aggressive bone tumorous lesion in infancy: osteofibrous dysplasia of the tibia and fibula. J Pediatr Orthop. 1993 Sep-Oct. 13(5):577-81. [Medline].

  15. Bridge JA, Dembinski A, DeBoer J. Clonal chromosomal abnormalities in osteofibrous dysplasia. Implications for histopathogenesis and its relationship with adamantinoma. Cancer. 1994 Mar 15. 73(6):1746-52. [Medline].

  16. Sherman GM, Damron TA, Yang Y. CD99 positive adamantinoma of the ulna with ipsilateral discrete osteofibrous dysplasia. Clin Orthop. 2003 Mar. 256-61. [Medline].

  17. Mandahl N, Heim S, Rydholm A. Structural chromosome aberrations in an adamantinoma. Cancer Genet Cytogenet. 1989 Oct 15. 42(2):187-90. [Medline].

  18. Sozzi G, Miozzo M, Di Palma S. Involvement of the region 13q14 in a patient with adamantinoma of the long bones. Hum Genet. 1990 Oct. 85(5):513-5. [Medline].

  19. Sakamoto A, Oda Y, Iwamoto Y, Tsuneyoshi M. A comparative study of fibrous dysplasia and osteofibrous dysplasia with regard to Gsalpha mutation at the Arg201 codon: polymerase chain reaction-restriction fragment length polymorphism analysis of paraffin-embedded tissues. J Mol Diagn. 2000 May. 2(2):67-72. [Medline].

  20. Gray MJ, Kannu P, Sharma S, et al. Mutations Preventing Regulated Exon Skipping in MET Cause Osteofibrous Dysplasia. Am J Hum Genet. 2015 Dec 3. 97 (6):837-47. [Medline].

  21. Anderson MJ, Townsend DR, Johnston JO. Osteofibrous dysplasia in the newborn. Report of a case. J Bone Joint Surg Am. 1993 Feb. 75(2):265-7. [Medline].

  22. Hindman BW, Bell S, Russo T. Neonatal osteofibrous dysplasia: report of two cases. Pediatr Radiol. 1996. 26(4):303-6. [Medline].

  23. Zamzam MM. Congenital osteofibrous dysplasia of the tibia, associated with pseudoarthrosis of the ipsilateral fibula. Saudi Med J. 2008 Oct. 29(10):1507-9. [Medline].

  24. Cetinkaya M, Ozkan H, Köksal N, Sarisözen B, Yazici Z. Neonatal osteofibrous dysplasia associated with pathological tibia fracture: a case report and review of the literature. J Pediatr Orthop B. 2012 Mar. 21(2):183-6. [Medline].

  25. Park YK, Unni KK, McLeod RA. Osteofibrous dysplasia: clinicopathologic study of 80 cases. Hum Pathol. 1993 Dec. 24(12):1339-47. [Medline].

  26. Van Delm I, Fabry G. Osteofibrous dysplasia of the tibia: case report and review of the literature. J Pediatr Orthop B. 1999 Jan. 8(1):50-3. [Medline].

  27. Campbell CJ, Hawk T. A variant of fibrous dysplasia (osteofibrous dysplasia). J Bone Joint Surg [Am]. 1982 Feb. 64(2):231-6. [Medline].

  28. Kahn LB. Adamantinoma, osteofibrous dysplasia and differentiated adamantinoma. Skeletal Radiol. 2003 May. 32(5):245-58. [Medline].

  29. Maki M, Athanasou N. Osteofibrous dysplasia and adamantinoma: correlation of proto-oncogene product and matrix protein expression. Hum Pathol. 2004 Jan. 35(1):69-74. [Medline].

  30. Dockerty MB, Meyerding HW. Adamantinoma of the tibia. JAMA. 1942. 119:932-37.

  31. Markel SF. Ossifying fibroma of long bone: its distinction from fibrous dysplasia and its association with adamantinoma of long bone. Am J Clin Pathol. 1978 Jan. 69(1):91-7. [Medline].

  32. Schajowicz F, Santini-Araujo E. Adamantinoma of the tibia masked by fibrous dysplasia. Report of three cases. Clin Orthop. 1989 Jan. (238):294-301. [Medline].

  33. Ueda Y, Blasius S, Edel G. Osteofibrous dysplasia of long bones--a reactive process to adamantinomatous tissue. J Cancer Res Clin Oncol. 1992. 118(2):152-6. [Medline].

  34. Czerniak B, Rojas-Corona RR, Dorfman HD. Morphologic diversity of long bone adamantinoma. The concept of differentiated (regressing) adamantinoma and its relationship to osteofibrous dysplasia. Cancer. 1989 Dec 1. 64(11):2319-34. [Medline].

  35. Springfield DS, Rosenberg AE, Mankin HJ. Relationship between osteofibrous dysplasia and adamantinoma. Clin Orthop. 1994 Dec. (309):234-44. [Medline].

  36. Gleason BC, Liegl-Atzwanger B, Kozakewich HP, Connolly S, Gebhardt MC, Fletcher JA, et al. Osteofibrous dysplasia and adamantinoma in children and adolescents: a clinicopathologic reappraisal. Am J Surg Pathol. 2008 Mar. 32(3):363-76. [Medline].

  37. Mathew M, Joseph B. Differentiated adamantinoma: a case report and review of literature. Indian J Pathol Microbiol. 2007 Jul. 50(3):565-7. [Medline].

  38. Hazelbag HM, Taminiau AH, Fleuren GJ. Adamantinoma of the long bones. A clinicopathological study of thirty- two patients with emphasis on histological subtype, precursor lesion, and biological behavior. J Bone Joint Surg Am. 1994 Oct. 76(10):1482-99. [Medline].

  39. Hatori M, Watanabe M, Hosaka M, Sasano H, Narita M, Kokubun S. A classic adamantinoma arising from osteofibrous dysplasia-like adamantinoma in the lower leg: a case report and review of the literature. Tohoku J Exp Med. 2006 May. 209(1):53-9. [Medline].

  40. Bethapudi S, Ritchie DA, Macduff E, Straiton J. Imaging in osteofibrous dysplasia, osteofibrous dysplasia-like adamantinoma, and classic adamantinoma. Clin Radiol. 2014 Feb. 69(2):200-8. [Medline].

  41. Grabias SL, Campbell CJ. Fibrous dysplasia. Orthop Clin North Am. 1977 Oct. 8(4):771-83. [Medline].

  42. Nakashima Y, Yamamuro T, Fujiwara Y. Osteofibrous dysplasia (ossifying fibroma of long bones). A study of 12 cases. Cancer. 1983 Sep 1. 52(5):909-14. [Medline].

  43. Sakamoto A, Oda Y, Iwamoto Y, Tsuneyoshi M. A comparative study of fibrous dysplasia and osteofibrous dysplasia with regard to expressions of c-fos and c-jun products and bone matrix proteins. Hum Pathol. 1999 Dec. 30(12):1418-26. [Medline].

  44. Most MJ, Sim FH, Inwards CY. Osteofibrous dysplasia and adamantinoma. J Am Acad Orthop Surg. 2010 Jun. 18(6):358-66. [Medline].

  45. Adler CP. Case report 587: Adamantinoma of the tibia mimicking osteofibrous dysplasia. Skeletal Radiol. 1990. 19(1):55-8. [Medline].

  46. Weiss SW, Dorfman HD. Adamantinoma of long bone. An analysis of nine new cases with emphasis on metastasizing lesions and fibrous dysplasia-like changes. Hum Pathol. 1977 Mar. 8(2):141-53. [Medline].

  47. Taylor RM, Kashima TG, Ferguson DJ, Szuhai K, Hogendoorn PC, Athanasou NA. Analysis of stromal cells in osteofibrous dysplasia and adamantinoma of long bones. Mod Pathol. 2012 Jan. 25(1):56-64. [Medline].

  48. Kuruvilla G, Steiner GC. Osteofibrous dysplasia-like adamantinoma of bone: a report of five cases with immunohistochemical and ultrastructural studies. Hum Pathol. 1998 Aug. 29(8):809-14. [Medline].

  49. Kanamori M, Antonescu CR, Scott M, et al. Extra copies of chromosomes 7, 8, 12, 19, and 21 are recurrent in adamantinoma. J Mol Diagn. 2001 Feb. 3(1):16-21. [Medline].

  50. Most MJ, Sim FH, Inwards CY. Osteofibrous dysplasia and adamantinoma. J Am Acad Orthop Surg. 2010 Jun. 18(6):358-66. [Medline].

  51. Kuruvilla G, Steiner GC. Osteofibrous dysplasia-like adamantinoma of bone: a report of five cases with immunohistochemical and ultrastructural studies. Hum Pathol. 1998 Aug. 29(8):809-14. [Medline].

  52. Ratra A, Wooldridge A, Brindley G. Osteofibrous Dysplasia-like Adamantinoma of the Tibia in a 15-Year-Old Girl. Am J Orthop (Belle Mead NJ). 2015 Oct. 44 (10):E411-3. [Medline]. [Full Text].

  53. Buldu H, Centel T, Kırımlıoğlu H, Dirik Y. Osteofibrous dysplasia-like adamantinoma in a 3-month-old male infant: a case report. Acta Orthop Traumatol Turc. 2015. 49 (2):210-2. [Medline].

  54. Wootton-Gorges SL. MR imaging of primary bone tumors and tumor-like conditions in children. Magn Reson Imaging Clin N Am. 2009 Aug. 17(3):469-87, vi. [Medline].

  55. Jung JY, Jee WH, Hong SH, Kang HS, Chung HW, Ryu KN, et al. MR findings of the osteofibrous dysplasia. Korean J Radiol. 2014 Jan-Feb. 15(1):114-22. [Medline]. [Full Text].

  56. Moretti VM, Slotcavage RL, Crawford EA, Lackman RD, Ogilvie CM. Curettage and Graft Alleviates Athletic-Limiting Pain in Benign Lytic Bone Lesions. Clin Orthop Relat Res. 2010 May 12. [Medline].

  57. Lee RS, Weitzel S, Eastwood DM, Monsell F, Pringle J, Cannon SR, et al. Osteofibrous dysplasia of the tibia. Is there a need for a radical surgical approach?. J Bone Joint Surg Br. 2006 May. 88(5):658-64. [Medline].

  58. Hahn SB, Kim SH, Cho NH, Choi CJ, Kim BS, Kang HJ. Treatment of osteofibrous dysplasia and associated lesions. Yonsei Med J. 2007 Jun 30. 48(3):502-10. [Medline].

  59. Mankin HJ, Trahan CA, Fondren G, Mankin CJ. Non-ossifying fibroma, fibrous cortical defect and Jaffe-Campanacci syndrome: a biologic and clinical review. Chir Organi Mov. 2009 May. 93(1):1-7. [Medline].

  60. Goergen TG, Dickman PS, Resnick D. Long bone ossifying fibromas. Cancer. 1977 May. 39(5):2067-72. [Medline].

  61. Ben Arush MW, Ben Arieh Y, Bialik V. Synovial sarcoma associated with osteofibrous dysplasia. A case report and review of the literature. Am J Pediatr Hematol Oncol. 1992 Aug. 14(3):261-4. [Medline].

  62. Huvos AG, Higinbotham NL, Miller TR. Bone sarcomas arising in fibrous dysplasia. J Bone Joint Surg [Am]. 1972 Jul. 54(5):1047-56. [Medline].

  63. Schwartz DT, Alpert M. The malignant transformation of fibrous dysplasia. Am J Med Sci. 1964 Jan. 247:1-20. [Medline].

  64. Witkin GB, Guilford WB, Siegal GP. Osteogenic sarcoma and soft tissue myxoma in a patient with fibrous dysplasia and hemoglobins JBaltimore and S. Clin Orthop. 1986 Mar. (204):245-52. [Medline].

Radiograph of osteofibrous dysplasia of tibia in 5-year-old girl.
Characteristic radiographic findings of osteofibrous dysplasia. Note eccentric intracortical lesion with sclerosis of internal surface, bubbled appearance of lesion, and anterior tibial bowing.
Typical histologic appearance of osteofibrous dysplasia lesion (×100). Note zonal architecture with periphery of active osteoblasts surrounding bone trabeculae.
Histologic section (×100) demonstrating vascular channels within osteofibrous dysplasia lesion, which has been proposed as etiologic factor in development.
Histologic appearance of fibrous dysplasia, revealing appearance similar to osteofibrous dysplasia but lacking periphery of active osteoblasts.
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