A case of maxillary odontogenic myxoma with tuberous sclerosis

Odontogenic myxoma (OM) is a relatively rare benign tumor of mesenchymal origin. It is characterized by slowly-growing, locally aggressive, and non-metastasizing neoplasms of the jaw bone. Tuberous sclerosis complex (TSC) develops as an abnormal growth of ectodermal and mesodermal cells, producing benign tumors that extend to areas of the head, heart, brain, and kidneys. The syndrome of tuberous sclerosis (TS) is characterized by the triad of epilepsy, intellectual disability, and facial angiofibromas. A 43-year-old Japanese woman who had been diagnosed as having TS visited our institute with a chief complaint of swelling on the left side of the upper jaw. We performed imaging examination and biopsy. The results of these investigations confirmed that the lesion was OM, and partial resection of the left maxilla was performed under general anesthesia. The relationship between OM and TS is not clear. However, many authors have identified two loci on different chromosomes as causative genes of TS. TSC1 and TSC2 genes are tumor suppressor genes of TS, and mutation of either can induce the development of more aggressive tumors. We report a rare case of OM with the patient of TS.