Multicentric familial squamous odontogenic tumor

Three cases of multicentric squamous odontogenic tumor, one of which was previously reported, are presented in three black siblings. A review of the literature indicates that 5 of the 26 previously recorded cases arose in multiple sites, but no evidence of familial history has been suggested. Although squamous odontogenic tumor has been described as a relatively rare, benign odontogenic neoplasm, the multicentric familial nature of our present cases suggests this represents either a reactive hyperplasia or hamartoma of residual odontogenic epithelium within the periodontal tissues. Management of previous cases has varied from simple curettage to maxillectomy with only one case of recurrence.