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.

Primary malignant melanoma of the oral mucosa.

PURPOSE: The occurrence of primary oral melanoma is very rare. Large clinical series suggesting appropriate treatment modalities are lacking; the clinician has to rely on case reports to gain insight into the management of this tumor, which is much more aggressive than its skin counterpart. Patients and methods The cases of 5 patients with primary oral melanoma during a 6-year period were retrospectively reviewed. Four patients presented with tumors located in the maxilla. Preoperative workup included microscopic examination of incisional biopsy specimens for the confirmation of the diagnosis and thorough imaging of the body to rule out distant metastases. Histologically significant vertical invasion was found in all patients. No patient had distant metastases initially, and only 1 patient presented with neck disease. Primary treatment included wide local excision in 4 patients and therapeutic modified radical neck dissection in the 1 patient with neck node involvement on presentation. One patient was treated primarily with radiation therapy due to medically compromised status. Adjuvant immunochemotherapy (DAV protocol) was administered to 4 patients. Radiation therapy was used in an adjuvant fashion in 3 patients. RESULTS: All of the patients developed distant metastases to the lung and liver and eventually died of their disease. Survival ranged from 14 to 38 months (mean, 25.6 months). Local recurrence occurred in 1 patient and was attributed to positive surgical margins. Contralateral neck disease developed in the patient who underwent therapeutic neck dissection. CONCLUSIONS: The prognosis of primary oral melanoma remains poor despite adequate locoregional control of the disease. It seems that diagnosis is made late in the course of the disease when the primary tumor has already shed microscopic distant metastases.