Bilateral keratocystic odontogenic tumor of mandible – A unique pediatric lesion: Case report and review

Abstract

The keratocystic odontogenic tumor (KCOT) is the most common cystic neoplasm in the maxillofacial region. Multiple odontogenic keratocyst are usually associated with Nevoid Basal Cell Carcinoma (NBCC) syndrome. A variant of this neoplasm is the sporadic multiple KCOT in pediatric population. Presented here is a case of bilateral keratocystic odontogenic tumor in a fourteen year old. Multiple keratocysts in the pediatric population is rare, aggressive, and recurrent and the first sign of NBCC or the solo presentation of human homologue of the PTCH (Drosophila segment polarity gene Patched) gene mutation.