Endovascular papillary angioendothelioma (Dabska tumor) of the tongue: report of a case

Endovascular papillary angioendothelioma (EPA), known as Dabska tumor, is a very rare vascular neoplasm that usually occurs in the skin or subcutis of infants and young children. There is no previous report of EPA appearing in the oral cavity. Here, we present an exceptionally rare case of EPA of the tongue in a 67-year-old man. A well-defined, reddish tumor measuring 11 mm x 8 mm x 7 mm existed at the submucosal area of the left tongue margin. Microscopic examination of an excisional biopsy specimen revealed the endothelioid tumor cells showing a papillary growth pattern, such as blood vessel-like structures. Immunohistochemical studies showed positive reactivities for CD31, CD34, alpha-smooth muscle actin, and factor VIII-related antigen in most of the tumor cells and CD68 in some tumor cells. Based on these observations, the tumor was diagnosed as an EPA.