Leiomyosarcomas of the oral tissues: clinicopathologic analysis of 50 cases.

PURPOSE: Primary oral leiomyosarcomas are rare tumors. Information regarding the biological behavior, prognosis, and appropriate management of this neoplasm is lacking in the literature. The purpose of this report was to summarize the data of isolated case reports of primary oral leiomyosarcoma that have been published in the English literature during the past 25 years. The cases of 4 additional new patients who have been treated in our department during the past 10 years are also presented. PATIENTS AND METHODS: The data for 46 patients obtained from 32 individual articles retrieved from the English literature were added to our 4 cases and produced a total number of 50 cases of primary leiomyosarcomas of the oral tissues. Patients were analyzed according to demographic data, anatomic location, type of treatment, and survival. RESULTS: Primary oral leiomyosarcoma may affect any age with peaks of occurrence in the third, sixth, and seventh decades of life. There is no gender predilection. Female patients presented the higher incidence in the third decade, whereas males had an even age distribution. The tumor arises in approximately 70% of the cases in the maxillary and mandibular bones. Radical surgery was the treatment of choice. Radiotherapy and chemotherapy when applied in recurrent tumors had little effect. The most adverse prognostic factor was the positive surgical margins. The 5-year survival was 62% (62.9% for females and 52.6% for males, P > .1968). CONCLUSIONS: Cases of oral leiomyosarcoma appear to be associated with major neurovascular structures of the facial skeleton, as evidenced from the imaging studies of our 4 patients. Primary oral leiomyosarcoma is a rare tumor that should be managed with aggressive surgical resection in order to safeguard curability. Histopathologic diagnosis is greatly facilitated with positive immunohistochemical staining for smooth muscle antigenic markers.