FaCD Online Syndrome Fact Sheet

Last updated: 22 Feb 2008

Name: Craniofacial Fibrous Dysplasia

Mode of Inheritance: AD?

Tumor features

maxillar/mandibular tumors, fibro-osseous

Non-tumor features

bone, fibrous dysplasia & sclerosis of
jaw, fibrous dysplasia of the


Pierce et al.[1] reported on a mother and two of her three daughters with fibrous dysplasia of the head and neck region. In that family the expansion of the facial lesions slowed or ceased in the early teenage years and became sclerotic. Craniofacial fibrous dysplasia is one of the four types of fibrous dysplasia (the three being: monostotic, polyostotic and polyostotic with skin lesions and endocrine dysfunction (McCune-Albright)). These types are all benign fibro-osseous conditions involving one or more bones. The authors emphasize that craniofacial dysplasia is distinct from cherubism (and also Ramon syndrome) : where intrabony jaw lesions are lytic and consist of fibrous tissue with giant cells.
Malignant transformation has been observed in fibrous dysplasia in general, often involving the craniofacial region.


[1] Pierce AM, Sampson WJ, Wilson DF, Goss AN. Fifteen-year follow-up of a family with inherited craniofacial fibrous dysplasia. J Oral Maxillofac Surg 1996; 54(6):780-788.