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osteofibrous dysplasia

Disease Summary
Associated Targets (1)
Tclin

1


GARD Rare
Mondo Description A benign, usually self-limited fibro-osseous lesion of the bone that affects infants and children. It usually arises from the cortical bone of the anterior mid-shaft of the tibia. Patients usually present with swelling or painless bowing of the tibia. Progression to adamantinoma has been reported in some cases.
Uniprot Description A congenital disorder of osteogenesis characterized by non-neoplastic, radiolucent lesions that affect the cortical bone immediately under the periosteum. It usually manifests as a painless swelling or anterior bowing of the long bones, most commonly the tibia and fibula.
Mondo Term and Equivalent IDs
MONDO:0011806:  osteofibrous dysplasia
MESH:C563276: 
NCIT:C53970: 
Orphanet:488265: 
UMLS:C1709353: