Epithelioid Hemangioendothelioma of Mandibular Gingiva: A Challenging Diagnosis

Epithelioid hemangioendothelioma (EHE) is a rare neoplasm derived from the vascular endothelium. Although it can occur anywhere in the body, few cases have been described in the oral cavity. We report a 47-year-old woman presenting with a painful ulcerated lesion on the mandibular gingiva, suggestive of a traumatic decubitus ulcer. Histology and immunohistochemistry confirmed the diagnosis of epithelioid hemangioendothelioma. A literature review of EHE of mandibular gingiva was done. Pubmed were searched from 1975 through June 2024 using the following search terms: epithelioid hemangioentothelioma, vascular tumor, oral cavity and mandibular gingiva. Relevant manuscripts were selected and the results were used to update a narrative overview of the diagnosis and management of this entity. We found 38 cases of EHE in the oral cavity, of which 16 were located on the gingiva. Most of them were located on the mandibular gingiva as painless swelling, unlike our case. 70 % of the cases presenting in the mandibular gingiva had bone resorption on imaging. However, only half of those located in the maxillary gingiva had this bone resorption. Only 2 cases located in the mandibular gingiva presented recurrence and 1 of them debuted with nodal metastases after a 7-year follow-up. The clinical and histological diagnosis of EHE is complex and must be confirmed by immunohistochemistry. Upon diagnosing this entity, we should perform an excision with clear margins and conduct long-term follow-up due to the risk of local and distant recurrence. Key words:Epithelioid hemangioendothelioma, Gingival pathologies, Oral cavity, Mandibular Diseases, CD31, Immmunohistochemical markers, Vascular tumor.