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Don’t fight Osteofibrous dysplasia alone.
Find your community on the free RareGuru App.Osteofibrous dysplasia is a rare, non-cancerous (benign) tumor that affects the long bones. It usually develops in children and adolescents. The most common location is the middle part of the tibia (shin), although the fibula (a smaller bone in the calf) and the long bones in the arm (humerus, radius, or ulna) may also be affected. In many cases, there are no symptoms and the condition is discovered when an x-ray is done for another reason (incidental finding). When symptoms are present, they most often include swelling and/or pain at the site of the tumor, a break in the bone (fracture) where it is weakened by the tumor, and/or bowing of the leg. The cause of osteofibrous dysplasia is unknown. Treatment is usually conservative, involving observation until the bone stops growing (skeletal maturity). Bracing may help prevent bowing of the limb and fractures. Surgery may be recommended once bone growth is complete.
Source: GARD Last updated on 05-01-20
The course of disease progression is variable. In some cases, the tumor grows at a moderate pace throughout childhood. In other cases, tumor growth is aggressive, resulting in bowing and deformity of the limb. Spontaneous resolution is also possible. Once the bone stops growing, the condition usually stabilizes or improves. If surgery is performed before the bone is done growing, recurrence is common (64-100%). Recurrence is unlikely after skeletal maturation. Malignant transformation has not been reported.
Last updated on 05-01-20
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