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Familial ossifying fibroma

Disease definition

A rare genetic bone disease characterized by multifocal, painless, benign fibrocemento-osseous lesions of the jaws which expand progressively and can cause severe facial deformity. It usually manifests at an early age and is often associated with abnormalities of the long bones and pathologic fractures. Radiologically, the lesions are of mixed radiopaque/radiolucent appearance. Incomplete surgical removal may lead to more rapid growth of the residual lesion.


Classification level: Disorder
  • Synonym(s):
    • Multiple ossifying fibroma
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood, Adolescent, Adult
  • ICD-10: D16.4
  • OMIM: 137575
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -
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