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.