Abstract
A low-grade fibromyxoid sarcoma (LGFMS) is an extremely rare tumor first described by Evans in 1987. LGFMS is a type of soft tissue sarcoma characterized by a deceptively benign histological appearance but completely malignant behavior. LGFMS is usually seen in the deep soft tissues of the extremities and trunk. We have examined many reviews, case reports and case series previously published in PubMed and Google Scholar. To date, only five cases have been reported in the maxilla. LGFMS generally affects young adults, but it can also be seen in children and older adults. A radical surgical approach is the most recommended treatment option. LGFMS has a very low mitotic activity; therefore, it is considered that neither chemotherapy nor radiotherapy has a significant effect on long-term LGFMS prognosis. However, to date, there has been no study suggesting any protocol for the follow-up of patients with LGFMS. In this report, we present a case with LGFMS located in the maxillary sinus, in which despite radiotherapy following extensive surgical excision, the tumor recurred in a short period of three months and reached its former size.