Title

Osteofibrous dysplasia and adamantinoma

UMMS Affiliation

Department of Orthopedics and Physical Rehabilitation

Date

6-1-2010

Document Type

Article

Medical Subject Headings

Adamantinoma; Bone Neoplasms; Diagnosis, Differential; Fibrous Dysplasia of Bone; Humans; Neoplasm Staging; Prognosis

Disciplines

Orthopedics | Rehabilitation and Therapy

Abstract

Osteofibrous dysplasia (OFD) is a rare, benign, fibro-osseous lesion that typically is seen within the cortex of the tibia in children. Adamantinoma (AD) is a rare, low-grade malignant primary bone tumor that occurs most often in the tibia and/or fibula of adolescent persons and young adults; however, it has been reported in other long bones, as well. Immunohistochemical and ultrastructural evidence has shown that the neoplastic cell in AD derives from an epithelial lineage. More recently, published reports have described another clinical entity-differentiated or OFD-like AD-that appears to lie between OFD and AD along a spectrum of disease. Controversy exists as to whether OFD is a precursor lesion to AD or whether OFD may be a residual lesion resulting from a spontaneously regressing AD. Management of OFD varies from observation to surgical intervention, depending on the age of the patient and the extent of the lesion. Management of AD requires surgical resection with wide margins, followed by appropriate reconstruction, to minimize the risk of local recurrence or metastasis.

Rights and Permissions

Citation: J Am Acad Orthop Surg. 2010 Jun;18(6):358-66.

Related Resources

Link to Article in PubMed

PubMed ID

20511441