Case report: A rare presentation of high risk epithelioid hemangioendothelioma on leg treated with surgical excision

INTRODUCTION: A rare vascular tumor, epithelioid hemangioendothelioma (EHE), can be difficult to diagnose for physicians. Although uncommon, EHE has the potential to become malignant and cause patient death. The five year mortality rate with the diagnosis of the high risk type has been reported to be as high as 41. Thus this finding requires aggressive treatment to prevent amputation or death (Deyrup et al., 2008). PRESENTATION OF CASE: In this case, a 60-year-old male patient was evaluated for a suspicious subepidermal nodule on the upper lateral aspect of the lower right leg just distal to the knee. It was excised to the level of muscle with appropriate margins of 1 cm circumferentially. The specimen underwent appropriate pathological testing and did reveal a high-risk-type epithelioid hemangioendothelioma with remaining tumor present at the deep margin. The patient had additional surgery by an orthopedic surgeon in order to obtain clear margins of the lesion and retain maximum function of leg. Despite surgical excision with ultimately clean margins, the lesion did metastasize to the right groin area seven months after surgery and the secondary metastatic site also required surgical excision. DISCUSSION: There is very little information available to the identification and treatment of a finding of EHE. The only definitive treatments to prevent malignancy is excision or amputation, possibly in conjunction with radiation therapy. The role of oncology intervention should be considered because the finding may be either a cause or an effect of malignancy. CONCLUSION: The goal of this paper is to raise awareness of the importance of pathology for soft issue lesions even if there is initially a low clinical index of suspicion. Unique characteristics in the pathology is the cornerstone to the identification and treatment. Although surgical excision can appear to be a curative treatment, EHE may inevitably metastasize, so aggressive and definitive treatment is best.