Epithelioid Hemangioendothelioma Arising from Interanl Jugular Vein Mimicking Cervical Metastatic Lymphadenopathy

Abstract

Epithelioid hemangioendothelioma (EHE) is a relatively rare and moderately aggressive type of vascular tumor. We describe a case of EHE that presented in a 24-year-old woman as a palpable nodule in the left cervical area. Radiological examinations and fine-needle aspiration cytology led to a preliminary diagnosis of metastatic lymphadenopathy with an unknown primary site. However, during surgery, we determined that the nodule was an intra-vascular tumor arising from the left internal jugular vein. The histopathological examination revealed cords of epithelioid endothelial cells distributed in a hyaline stroma, compatible with a diagnosis of EHE.

Keywords: Epithelioid hemangioendothelioma; Pathology; Vascular disease.

Fig. 1

Chest computed tomography scan shows a 3-cm mass (arrow) in the left supraclavicular area with a markedly narrowed left internal jugular vein. (A) Coronal view. (B) Axial view. (C) A positron emission tomography scan shows increased uptake (maximum standardized uptake value of 7.7) in the left supraclavicular area.

Fig. 2

Histopathological and immunohistochemical findings. (A) Cords and nests of spindled epithelioid cells with intracytoplasmic vacuoles were observed in a hyalinized stroma (H&E, ×100). The tumor cells were immunopositive for (B) vimentin (×200), (C) CD31 (×200), and (D) CD34 (×200).