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I borrowed this clip from another example for fibrous dysplasia on the site ( [Ref #1]). Fibrous dysplasia is a benign developmental disorder of bone. These proliferative fibro-osseous lesions can be either monostotic (80%) or polyostic (20%). Patients with monostotic disease range in age from infancy to greater than 70 years of age, with the majority of patients being diagnosed between 5 and 20 years of age. One third of monostotic lesions involve the craniofacial bones, one third involve the femur or tibia, and about 20% involve the ribs. Clinical presentation varies with the site of involvement. Craniofacial disease usually produces obvious deformities with minimal to no symptoms (some cases in the jaw present with dental malocclusion). Long bone disease may present with pain following fracture. Rib lesions are typically asymptomatic, incidental findings. Patients with polyostotic fibrous dysplasia can have associated pigmented skin lesions and endocrine disorders; this association characterizes McCune-Albright syndrome. Rarely, these patients can have associated fibromyxomatous soft tissue tumors; this constellation is known as Mazabraud’s syndrome. Polyostotic disease usually manifests itself before age 10 with limp, pain or fracture owing to abnormal skeletal development. The femur, tibia and pelvis are involved in 75-90% of patients with polyostotic disease. Microscopically, the biopsy demonstrates irregular trabeculae of woven bone (classically described as Chinese character arrangements) without osteoblastic rimming, surrounded by a relatively cellular fibrous connective tissue.

----Comment by: PathDoc15 on 6/2/2009 12:58:50 PM


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