National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Osteofibrous dysplasia


Other Names:
Ossifying fibroma; Intracortical fibrous dysplasia; Jaffe-Campanacci syndrome
Categories:
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.[1][2] 
Last updated: 10/20/2016

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
5%-29% of people have these symptoms
Fibular hypoplasia
Short calf bone
0003038
Pectus excavatum
Funnel chest
0000767
Percent of people who have these symptoms is not available through HPO
Autosomal dominant inheritance 0000006
Pseudoarthrosis 0005864
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Last updated: 7/1/2020
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.[1] Malignant transformation has not been reported.[2]
Last updated: 10/20/2016

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Osteofibrous dysplasia. Click on the link to view a sample search on this topic.

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  1. Davidson D. Osteofibrous Dysplasia. Medscape. March 11, 2016; http://emedicine.medscape.com/article/1256595-overview.
  2. Osteofibrous Dysplasia and Adamantinoma. American Academy of Orthopaedic Surgeons (AAOS). June 2014; http://orthoinfo.aaos.org/topic.cfm?topic=A00685.