Primary epithelioid hemangioendothelioma of the head and neck region: a systematic review of the last 38 years

Hemangioendothelioma is a vascular neoplasm with potentially aggressive local behavior, commonly seen in soft tissues, bones, and lungs. Although uncommon, head and neck region can be affected, with emphasis on the gingiva and tongue. In general, three histological presentation forms are recognized: kaposiform, retiform, and epithelioid. The epithelioid variant is characterized by the presence of endothelial cells with epithelioid morphology. Head-neck Epithelioid hemangioendothelioma is a rare lesion in which there are few case studies seen in the literature. Thus, the objective of the present study is to perform a systematic review on head-neck epithelioid hemangioendothelioma, with emphasis on evaluating the expression of the chemical markers CD31 and CD34. To this end, studies were selected from the Embase, PubMed, Scopus and Web of Science databases using the keywords “Epithelioid hemangioendothelioma” and “Oral epithelioid hemangioendothelioma”. Boolean operators (“AND” and “OR”) were used to improve search capability. Subsequently, two evaluators screened the articles initially selecting the titles and abstracts, and then, the full text of the study was screened. Any disagreement between the evaluators were resolved by a third evaluator. The present study presents data regarding demographic and clinicopathological of EHE: the average age was 41.2 years, with higher prevalence in females, mainly affected injury sites were the gingiva (24.44%), and the most present chemical markers were CD31 and CD34, both with 64.52% of the cases. The present study provides important information that can assist dentists and pathologists in diagnosis, and subsequently assisting in the treatment plan for head-neck EHE.