Abstract

A 17-year girl reported with painful right lower posterior teeth. Orthopantomogram showed unilocular radiolucency with scalloped non-sclerotic border at apical area of non-carious right mandibular molars and premolar. A provisional radiological diagnosis of ameloblastoma or odontogenic keratocyst was given. Histopathological examination revealed follicular areas of peripheral palisaded hyperchromatic basaloid cells and central round-polygonal clear cells. A diagnosis of clear cell odontogenic carcinoma (CCOC)-ameloblastomatous variant was made after assessing the provisional diagnoses. A nosological dilemma arose as many authors opined that the terms ‘clear cell ameloblastoma’ and ‘clear cell odontogenic tumor’ should be invalidated and CCOC should be the preferred diagnosis because of the reported aggressive nature of clear cell odontogenic neoplasms. The scientific literature gave variable biological behavior and prognosis with diverse therapeutic approaches leading to therapeutic dilemma in management of the case. The authors have attempted to resolve the diagnostic and therapeutic challenges by presenting the clinical, radiological and histological aspects of the case and discussing the differential diagnoses of clear cell lesions involving the maxillofacial region along with the therapeutic approaches and prognosis of CCOC.

Keywords: Clear cell; Odontogenic; Carcinoma; Tumor; Ameloblastoma; Metastatic; Salivary gland; CCOC; CCOT