Osteofibrous Dysplasia Workup

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

Laboratory Studies

  • Laboratory studies typically are not necessary in the diagnosis of osteofibrous dysplasia.
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Imaging Studies

  • Radiographs: In children, osteofibrous dysplasia initially engenders tremendous concern among clinicians and parents regarding the possibility of malignancy. However, the appearance usually is typical, to the extent that radiologic diagnosis generally is sufficient.
    • The radiographic appearance of osteofibrous dysplasia is characteristic, as seen in the image below, and has been reported in numerous studies. Characteristic radiographic findings of osteofibroCharacteristic 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.
    • Lesions are eccentric, intracortical, and osteolytic.
    • Variable expansion of the external cortical surface is present, with sclerosis of the internal cortical surface.
    • Frequently, a multilocular lesion will give rise to a bubbled appearance.
    • Soft-tissue extension is absent, and periosteal reaction is rare, unless there is an associated pathologic fracture.
    • The size of the lesion is variable. Usually, it affects the diaphysis, although metaphyseal encroachment has been reported.
  • To date, diagnostic characteristics of osteofibrous dysplasia with CT scans or MRI have not been reported.[35]
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Diagnostic Procedures

  • Biopsy
    • Because the clinical course and radiologic appearance of osteofibrous dysplasia is diagnostic in children, biopsy is seldom 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. If a biopsy is performed, histologic examination is generally definitive.
    • If biopsy is necessary to confirm the diagnosis, consult with a radiologist and a pathologist to ensure an adequate specimen.
    • Adhere to strict biopsy principles, as a malignant process has not yet been excluded.
    • Biopsy the tibia away from the apex of the tibial curvature to minimize the development of a fatigue fracture, which is common following biopsy in osteofibrous dysplasia.
    • Incise the skin longitudinally and minimize dissection to the greatest extent possible. Disrupt as few compartments as possible; dissect through, rather than adjacent to, muscle; fill bone defects; and strictly maintain hemostasis.
    • Biopsy material should include periosteum, cortical bone, and medullary material, both central and peripheral to the lesion.
    • Tissue obtained must be representative of the lesion and adequate for histologic grading. Obtain a frozen section to ensure the specimen is sufficient.
    • Avoid leaving sharp edges that may act as stress risers, leading to postbiopsy fracture.
    • After biopsy, protect the limb in a cast or splint for 3-6 weeks.
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Histologic Findings

Despite the characteristic radiographic appearance, Wang et al recommended that diagnosis should be based on biopsy and pathologic examination, as seen in the image below.[6] At the time of surgery, inspection reveals an intact periosteum. The cortex is thinned and may be perforated. The lesion itself is composed of soft, granular tissue that is whitish-yellow in color.

Typical histologic appearance of the lesion under Typical histologic appearance of the lesion under 100X magnification. Note the zonal architecture with a periphery of active osteoblasts surrounding bone trabeculae.

Histologic characteristics of osteofibrous dysplasia have been described well in the literature. The overall appearance is that of zonal architecture. The lesion is fibrous at its center, with immature woven bone trabeculae. Vascular channels have been described within the lesion, as seen in the first image below. At the periphery, a prominent border of active osteoblasts rims the bony trabeculae. The presence of such a border is a differentiating factor between osteofibrous dysplasia and fibrous dysplasia, in which there is no border of active osteoblasts, as seen in the second image below.

Histologic section under 100X magnification demonsHistologic 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 revealiHistologic appearance of fibrous dysplasia revealing a similar appearance to osteofibrous dysplasia but lacking the periphery of active osteoblasts

As examination proceeds from the center of the lesion to the periphery, the bone trabeculae become larger and more lamellar in appearance. Fibroblasts in the lesion have been noted to be well-differentiated. Several studies have reported cytokeratin-positive elements on immunohistochemical staining. Occasional hemorrhagic zones, cysts, or foci of cartilaginous differentiation have been reported. Multinucleated giant cells have also been observed.

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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.

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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
 
 
 
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