Fibrous dysplasia: a systematic review

To evaluate the principal features of fibrous dysplasia (FD) by systematic review (SR) and to compare their frequencies between four global groups.

The databases searched were the PubMed interface of Medline and LILACS. Only those reports of FD which occurred in a series in the reporting authors' caseload were considered. All cases were confirmed fibro-osseous lesions histopathologically. The SR-included series must also have included radiographs.

Of the 106 reports considered (including the Hong Kong report), 31 reports and a total of 788 cases were included in the SR. 11 SR-included series were in languages other than English. FD affected both genders equally, but was 50% more prevalent in the maxilla. The mean age at first presentation was 24 years. The decade with the greatest frequency was the second, in which males accounted for 63%. The main symptom in 90% of all SR-included cases was swelling (including deformation of the jaws). Not one SR-included case directly involved the ocular apparatus. All cases displayed buccolingual expansion; all mandibular cases exhibited downward displacement of the lower border of the mandible and almost all maxillary cases involved the maxillary antrum. Only 35% of reports included follow-up; 18% of cases recurred or were reactivated. Not one case displayed sarcomatous change.

Long-term follow-up of large series that would have revealed the long-term outcomes of FD was lacking. This is necessary because many cases do not burn out at the end of adolescence, as expected of a hamartoma, but are reactivated.