A Rare Epithelioid Hemangioendothelioma of the Tongue: A Case Report and Review of Published Cases

Aim: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor caused by the proliferation of endothelial cells. The purpose of this study was to describe a rare case of EHE of the tongue and to perform a systematic review of the literature reporting oral EHE. Methods: A 58-year-old woman was referred to our Oral Medicine Department for evaluation of a slowly growing ulcerative lesion of the tongue. Her medical history was unremarkable. Intraoral examination revealed a soft ulcerated mass with hypertrophic margins and a yellowish base located on the right lateral border of the tongue, measuring ~2 cm × 3 cm. An incisional biopsy was performed, and the histological exam revealed hyperplastic epithelium, a site of proliferation of epithelioid endothelial elements. Immunohistochemistry showed positivity for ERG, and a final diagnosis of EHE was made. After excisional surgery, no local recurrence was reported at 24 months’ follow-up. A systematic review was conducted on the PubMed database, following PRISMA guidelines. Results: Among 35 included studies, 44 cases of EHE were identified, including our case report (21 male and 21 female), with a mean age of 35 years (±19). The most frequently affected site was the gingiva, followed by the tongue (18 and 11). Local recurrence after surgical excision was reported in 18% of cases (8/44). Only one case of metastasis was documented. Conclusion: Vascular tumors should be considered in the differential diagnosis of soft tissue swellings of the oral cavity. Patients diagnosed with EHE require long-term follow-up due to the risk of local recurrence and the tumor’s uncertain malignant potential.