Osteofibrous Dysplasia Treatment & Management

  • Author: Robert Mervyn Letts, MD, FRCS(C), FACS; Chief Editor: Harris Gellman, MD   more...
 
Updated: Mar 1, 2012
 

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.

Next

Surgical Therapy

A characteristic of this lesion is the high recurrence rate following resection and curettage.[36] Thus, many authors advocate nonoperative treatment of the lesion until after skeletal maturity is reached, at which time marginal resection and bone grafting may be performed without increased risk of recurrence.[37, 38]

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.[3, 39] 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.

Previous
Next

Complications

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 following attempts at resection.[40] Wang et al also reported multiple recurrences following surgical intervention.[6] Campanacci and Laus indicated that recurrence does not occur in patients older than 10 years.[3]

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.[41] At the time of the latter diagnosis, CT scan confirmed multiple pulmonary metastases. Malignant transformation to soft-tissue sarcoma has been reported in fibrous dysplasia, most commonly in the polyostotic variation.[42, 43, 44] However, the case reported by Ben Arush et al is the only report of sarcomatous degeneration of osteofibrous dysplasia.

Previous
Next

Outcome and Prognosis

The natural history of osteofibrous dysplasia is unpredictable.[45] The growth rate can vary from slow to rapid, and spontaneous resolution is possible. Campanacci and Laus reported 3 common clinical courses: (1) moderate progression, particularly during the first 5-10 years of life; (2) aggressive growth, with resulting marked deformity; and (3) spontaneous resolution. Most commonly, there is continued growth of the lesion until skeletal maturity is reached, with the most rapid period of growth occurring before age 10 years. In most cases, moderate progression is followed by gradual improvement once skeletal maturity is attained.

Previous
Next

Future and Controversies

Osteofibrous dysplasia and adamantinoma have similar clinical presentations, as well as similar radiologic and pathologic findings. Although adamantinoma can sometimes have the appearance of a low-grade osteogenic sarcoma, osteofibrous dysplasia does not exhibit histologic characteristics of malignancy. There may be histologic gradations between osteofibrous dysplasia, benign adamantinoma, and the malignant appearance of more aggressive adamantinoma, which usually is encountered in adults. In the latter, osteoid production with cellular mitoses may give the appearance of an osteogenic sarcoma and, indeed, may progress to frank malignancy.

Because the clinical course and radiologic appearance of osteofibrous dysplasia are diagnostic in children, biopsy seldom is indicated and should be avoided, if possible. In patients presenting at skeletal maturity, in whom the incidence of adamantinoma is higher, biopsy of the mid portion of the lesion may be necessary for diagnosis. Complete resection of the entire lesion of osteofibrous dysplasia is neither recommended nor necessary.

Several authors have investigated the possible relationship between adamantinoma and osteofibrous dysplasia.[46, 47] Dockerty and Meyerding first reported a relationship between benign fibro-osseous lesions and adamantinoma.[48] Markel was the first to investigate this relationship thoroughly.[49] Subsequently, 3 cases of tibial adamantinoma that mimicked osteofibrous dysplasia were reported, 2 of which occurred in children younger than 10 years.[50]

Several investigators have proposed that osteofibrous dysplasia represents a benign form of adamantinoma or that it is the result of a resolved adamantinoma.[51] Czerniak et al described an intracortical lesion with pathologic findings similar to those of osteofibrous dysplasia, which they termed differentiated adamantinoma.[52] Further, they described differentiated adamantinoma as affecting individuals younger than those with classic adamantinoma. Czerniak et al and Springfield et al reported that differentiated or osteofibrous dysplasia-like adamantinoma can progress to adamantinoma.[52, 53] Thus, these lesions may represent intermediates in a continuum from osteofibrous dysplasia to adamantinoma.[54, 55]

Hazelbag et al reported several findings that support this relationship[56] :

  • First, they noted continuity from epithelial cells in osteofibrous dysplasia to primary epithelioid tumor, as in adamantinoma.
  • Second, the mean age at diagnosis of osteofibrous dysplasia and osteofibrous dysplasia-like adamantinoma is younger than the mean age at diagnosis of adamantinoma.
  • Third, there are similar radiographic findings.
  • Fourth, 2 patients in their study demonstrated progression from osteofibrous dysplasia to adamantinoma at the time of local recurrence. It has also been suggested that the sequence may occur in reverse, such that an adamantinoma may regress to osteofibrous dysplasia. However, Springfield et al disputed this claim and indicated that such regression is not likely.[53]

Findings supporting a relationship are in conflict with an investigation by Park et al, who reported no progression from osteofibrous dysplasia to adamantinoma and who contend that osteofibrous dysplasia is distinct from adamantinoma.[16] They did, however, suggest that osteofibrous dysplasia might be related to fibrous dysplasia, because 2 cases in their series transformed from osteofibrous dysplasia to monostotic fibrous dysplasia.

Several reports on the pathology of adamantinoma have shown that the lesion may have areas similar in appearance to osteofibrous dysplasia.[57] This finding suggests the potential for misdiagnosis in cases of inadequate biopsy. Such misdiagnosis may explain the reports of progression of osteofibrous dysplasia to adamantinoma. Thus, Springfield et al suggested that histologic diagnosis of osteofibrous dysplasia should be regarded with caution.[53] Hazelbag et al advocated biopsy of the center of the lesion to avoid such an error, while Sweet et al suggested examination of the entire specimen to identify areas consistent with adamantinoma.[2, 56]

Previous
 
Contributor Information and Disclosures
Author

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.

Coauthor(s)

Darin Davidson, MD  Resident, Department of Orthopedics, University of British Columbia

Disclosure: Nothing to disclose.

Specialty Editor Board

Lynn A Crosby, MD, FACS  Chief of Shoulder Division, Professor, Department of Orthopedic Surgery, Wright State University School of Medicine

Lynn A Crosby, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, American Academy of Orthopaedic Surgeons, American College of Sports Medicine, American College of Surgeons, American Fracture Association, American Medical Association, American Medical Tennis Association, American Orthopaedic Association, American Orthopaedic Foot and Ankle Society, Arthroscopy Association of North America, Mid-America Orthopaedic Association, and Orthopaedic Research Society

Disclosure: Nothing to disclose.

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

Disclosure: Medscape Salary Employment

Sean P Scully, MD, PhD  Professor, Department of Orthopedics, University of Miami

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

Disclosure: Nothing to disclose.

Dinesh Patel, MD, FACS  Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital

Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons

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

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, and Arkansas Medical Society

Disclosure: Nothing to disclose.

References

  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. Oct 2003;32(10):479-86. [Medline].

  2. 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. Mar 1992;16(3):282-90. [Medline].

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

  4. 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].

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

  6. 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. May 1992;(278):235-43. [Medline].

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

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

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

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

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

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

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

  14. 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. Mar 2012;21(2):183-6. [Medline].

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

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

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

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

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

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

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

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

  23. 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. May 2000;2(2):67-72. [Medline].

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

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

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

  27. 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. Dec 1999;30(12):1418-26. [Medline].

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

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

  30. 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. Mar 1977;8(2):141-53. [Medline].

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

  32. 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. Feb 2001;3(1):16-21. [Medline].

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

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

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

  36. 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. May 12 2010;[Medline].

  37. 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. May 2006;88(5):658-64. [Medline].

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

  39. 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. May 2009;93(1):1-7. [Medline].

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

  41. 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. Aug 1992;14(3):261-4. [Medline].

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

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

  44. 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. Mar 1986;(204):245-52. [Medline].

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

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

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

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

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

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

  51. 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].

  52. 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. Dec 1 1989;64(11):2319-34. [Medline].

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

  54. 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. Mar 2008;32(3):363-76. [Medline].

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

  56. 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. Oct 1994;76(10):1482-99. [Medline].

  57. 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. May 2006;209(1):53-9. [Medline].

  58. Cohen DM, Dahlin DC, Pugh DG. Fibrous dysplasia associated with adamantinoma of the long bones. Cancer. May-Jun 1962;15:515-21. [Medline].

  59. Jacobsen HG. Section III, case 14, In: Bone Disease (second series) Syllabus. Chicago: Waverly Press;1976: 233.

  60. Kitagawa T, Hayashi Y, Kimura O. [Osteogenic fibroma]. Seikei Geka. Oct 1968;19(11):906-12. [Medline].

  61. Schoenecker PL, Swanson K, Sheridan JJ. Ossifying fibroma of the tibia. Report of a new case and review of the literature. J Bone Joint Surg [Am]. Mar 1981;63(3):483-8. [Medline].

  62. Stephenson RB, London MD, Hankin FM. Fibrous dysplasia. An analysis of options for treatment. J Bone Joint Surg [Am]. Mar 1987;69(3):400-9. [Medline].

  63. Stewart MJ, Gilmer WS, Edmonson AS. Fibrous dysplasia of bone. J Bone Joint Surg Br. May 1962;44-B:302-18. [Medline].

  64. 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. Jan 2012;25(1):56-64. [Medline].

 
Previous
Next
 
Radiograph of osteofibrous dysplasia of the tibia in a 5-year-old girl
Characteristic radiographic findings of osteofibrous dysplasia. Note the eccentric intracortical lesion with sclerosis of the internal surface, bubbled appearance of the lesion, and anterior tibial bowing.
Typical histologic appearance of the lesion under 100X magnification. Note the zonal architecture with a periphery of active osteoblasts surrounding bone trabeculae.
Histologic section under 100X magnification demonstrating vascular channels within the lesion, which has been proposed as the etiologic factor in the development of the lesion
Histologic appearance of fibrous dysplasia revealing a similar appearance to osteofibrous dysplasia but lacking the periphery of active osteoblasts
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.