Epithelioid hemangioendothelioma of the oral cavity

Abstract

To investigate the clinicopathological characteristics and biologic behaviour of epithelioid hemangioendothelioma in the oral cavity. The clinical features and pathological findings of nine cases with intraoral epithelioid hemangioendothelioma were reviewed, including immunohistochemistry study. This series comprised seven males and two females aged 6-53 years (mean 28 years). The sites of the tumour included the tongue (n = 4), lip (n = 1), the gingiva and alveoli of the maxilla (n = 1), the gingiva and alveoli of the mandible (n = 1), buccal mucosa (n = 1), and the floor of the mouth (n = 1). A painless solitary mass was the most common presentation and was found in eight cases. On pathology, the tumour grew in short strands, cords or nests of polygonal to slightly spindled epithelioid cells in fibro-myxoid stroma, with formation of intracytoplasmic lumina. Tumour cells were immunoreactive to CD34, FVIIIRAg, and vimentin. Focal-positive cytokeration were observed in three cases. Immunoreactivity for S-100 protein, epithelial membrane antigen (EMA) and human herpesvirus (HHV)-8 was negative in all cases. Two cases recurred after surgical excision, but no patient developed local or distant metastasis. Wide local excision with long-term follow-up seems to be the treatment of choice for intraoral epithelioid hemangioendothelioma because of their unpredictable biological behaviour and recurrence potential.