Hemangioendothelioma is a rare, locally aggressive vascular tumor, originating from soft tissue, bone, skin, and organs such as liver or lung and exceptionally the oral cavity. It is usually benign but can show variable grades of malignancy. Most of the cases of oral epithelioid hemangioendotheliomas are asymptomatic, and diagnosis is hampered by the fact that the histological features somewhat overlap with hemangioma and angiosarcoma, with epithelioid cells, intracytoplasmic vacuoles, low mitotic activity, and rarely necrosis. Immunohistochemical analysis is required to rule out carcinoma or other epithelioid vascular neoplasms. Here, we report a very rare case of oral haemangioendothelioma in a 41-year female patient which presented as an exophytic, painless, mass on the labial gingiva, clinically diagnosed as pyogenic granuloma, which presented a diagnostically challenging histological picture, which required immunohistochemistry to achieve at the correct diagnosis.
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