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Angiosarcoma is a rare malignant tumor of endothelial origin. It is an aggressive neoplasm with early metastasis and poor prognosis and accounts for approximately 2% of all soft tissue sarcomas.Primary tumors arising in the oral cavity account for only 1% of all angiosarcomas. Here, we report a rare case of metastatic angiosarcoma of the gingiva originating from a primary mediastinal lesion. The patient was an 83-year-old man who presented with a maxillary interincisor tumor; it was a painless mass with rounded superficial necrosis measuring 23 mm × 17 mm on the labial side and 20 mm × 17 mm on the palatal side. The histopathological diagnosis was epithelioid angiosarcoma. Imaging revealed lesions in the mediastinum, lungs, liver, and skin. The primary lesion was considered a mediastinal lesion. As the tumor had spread throughout the body, palliative therapy was administered. However, the patient’s general condition deteriorated rapidly, and he died 3 weeks after the first visit. Diagnosis of oral metastatic malignancy may result in detection of malignant tumors at other sites. Therefore, it is important to detect and treat oral metastatic tumors early for long-term prognosis.

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