Spindle Cell Sarcoma of the Maxilla: A Rare Entity, Case Report, and Review of the Literature

Spindle cell sarcoma (SCS) is a malignancy, with the most recent Surveillance, Epidemiology, and End Results (SEER) data citing a total of 250 reported cases occurring in the head and neck. Of these cases, none originated in the maxillofacial hard tissue. To the best of our knowledge, only 2 cases of primary osseous SCS of the maxillofacial region have been reported. These cases were not accounted for in the SEER data. The diagnosis of SCS requires its differentiation from other sarcomas and spindle cell neoplasms. Therefore, a comprehensive review to reinforce its inclusion in oral and maxillofacial surgeons' differential diagnosis for osseous neoplastic pathology is desired. In the present case report, we have described a maxillary SCS in a patient with an initial diagnosis of a spindle cell lesion of uncertain biologic behavior. We reviewed the data for SCS, including the epidemiologic data, diagnostic challenges, clinical and radiographic presentations, prognostic indicators, and treatment.