Epithelioid hemangioendothelioma of the tongue: a report of solitary and multiple lesions in two young children

Abstract

Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor that typically involves the soft tissues, skin, viscera, bone, and, in rare cases, the oral cavity. Two extremely rare cases of EHE arising in the tongue of two young children are reported. One case developed as a solitary nodule on the lateral border of the tongue of a one-year-old boy, and the other case developed as multiple discrete small nodules on the dorsum of the tongue of a 3-year-old girl. The tumors were composed of epithelioid eosinophilic cells with intracytoplasmic vacuoles containing erythrocytes. Vascular differentiation of the tumors was confirmed by immunohistochemistry for such endothelial markers as CD31, von Willebrand factor, and Ulex europaeus agglutinin type I lectin (UEA-I) binding. Both cases were treated by wide local excision and showed no local recurrence during their long follow-up period. In conclusion, EHE can develop in very young children and can present itself as multiple intraoral lesions.