Inferior vena cava epithelioid hemangioendothelioma

Epithelioid hemangioendotheliomas are rare endothelial tumors that may manifest in a multitude of anatomic locations. The inferior vena cava (IVC) is an unusual site of primary tumor, with only two cases reported to date. We present a patient with an IVC epithelioid hemangioendothelioma who required IVC resection and lymphadenectomy. The patient made a good recovery and remains disease-free 4 years after resection. IVC tumors represent a unique challenge, not the least because of this tumor's unpredictable and, at present, undefined behavior, but also owing to the vital anatomic structures requiring definitive surgical excision.