ABSTRACT
Central granular cell odontogenic tumors (CGCOTs) are rare, benign, slowly growing odontogenic neoplasms. Due to their uncertain histogenesis, CGCOTs are still not included as a distinct entity in the WHO classification (2017) of odontogenic tumors. We report a case of CGCOT involving the right side of maxillary anterior region of a 39-year-old white female. Immunohistochemical staining showed that granular cells positively expressed CD68 and vimentin, and negatively expressed S-100 protein. Meanwhile, we searched PubMed, Google Scholar, and Scopus databases to summary the clinico-pathological features of 51 reported cases of CGCOT. The results showed that the granular cells of 28.6% cases were immunopositive for vimentin and CD68, and odontogenic epithelial cells were positive immunoreactivity for cytokeratin. These findings reinforced the mesenchymal origin of granular cells and the odontogenic nature of epithelium islands.