Osteofibrous Dysplasia

Excerpt

Osteofibrous dysplasia (OFD) is a benign fibro-osseous developmental condition of bone which commonly occurs in the cortical bone of the anterior mid-shaft of the tibia in children. First described by Frangenheim in 1921, it is also called congenital fibrous dysplasia and ossifying fibroma of the long bones.

The term "osteofibrous dysplasia of the tibia and fibula" was coined by Camanacci and Laus, thereby including the histological similarity to fibrous dysplasia and the occurrence of the disease in the tibia in the term. However, lesions may also affect the fibula, the radius, and the ulna. Osteofibrous dysplasia should be distinguished from adamantinoma, which is a malignant biphasic tumor characterized by different morphological patterns, clusters of epithelial cells, surrounded by a spindle-cell.

OFD can be classified as monostotic, polyostotic, and McCune Albright syndrome. Most cases of monostotic lesions present with no significant symptoms and are often found incidentally on x-ray. The condition mainly affects patients in their third decade.