Central adenoid cystic carcinoma of the mandible: case report and literature review of 16 cases.

Central intraosseous adenoid cystic carcinoma (ACC) of the mandible, formerly known as cylindroma, is a rare neoplasm with only 16 cases reported in the literature. We describe the diagnosis, etiology, and treatment of a central ACC located in the mandibular premolar region. We also review the literature. This case illustrates 2 key facts regarding the diagnosis and etiology of ACC. First, central salivary gland tumors should be considered in the differential diagnosis of cystic lytic lesions in the mandible. Second, even though the origin of this type of tumor is still unknown, the presence of ectopic tissue anterior to the submandibular gland in the submandibular area indicates that this tumor might be made up of ectopic embryogenic inclusions.