Epithelioid hemangioendothelioma is a vascular neoplasm that typically develops in soft tissue, lung, and liver and is extremely rare in the oral region. We report a case of epithelioid hemangioendothelioma of the incisive papilla region. A 62-year-old woman with a sessile, dark violet mass in the right side of the incisive papilla region visited our hospital. Orthopantomography and occlusal radiography showed no abnormal findings. These clinical findings strongly suggested epulis hemangiomatosa. Therefore, an excisional biopsy was performed under local anesthesia. However, histological examination revealed round or polygonal tumor cells with scattered mitotic figures, suggesting epithelioid hemangioendothelioma. Then, an additional resection with a sufficient safety margin and extraction of both maxillary central incisors were performed under general anesthesia. Immunohistochemical staining of the excisional biopsy specimen showed tumor cells positive for Factor VIII related antigen, CD31, and CD34, which are vascular endothelioma markers. Based on these histopathological findings, the final diagnosis was epithelioid hemangioendothelioma. No tumor cells were detected in the additionally resected specimen. Follow-up examinations have shown no recurrence for 5 years 1 month since the operation.
