Abstract
Hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare malignancy of vascular origin. When most patients with HEHE are diagnosed, they have a diffuse nodular phenotype, which is usually unresectable. A single nodular phenotype is found in only a small proportion (13%) of patients, and most are reported to be located in the right lobe of the liver. Although the prognosis of HEHE is considered more favorable than that of other hepatic malignant tumors, the 5-year survival rate is reported to be 64% after treatment. Herein, we present an unusual case of resectable HEHE of a single nodular type in the left lobe of the liver. The patient survived 15 years without recurrence after the multimodal treatment of radical resection and postoperative chemotherapy with thalidomide. The aim of the present study is to emphasize that multimodal treatment of radical resection followed by chemotherapy with thalidomide may achieve a relatively good survival outcome in patients with resectable HEHE of a single nodular type.
Highlights
Hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare malignancy of vascular origin
The single nodular type is found in only a minor proportion (13%) of patients, and most single tumors are reported to be situated in the right lobe of the liver [5]
Epithelioid hemangioendothelioma is a rare vascular tumor that may occur at various sites such as the skin, bone, stomach, and spleen and was originally described by Weiss and Enzinger in 1982 [8]
Summary
Abstract: Hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare malignancy of vascular origin. The prognosis of HEHE is considered more favorable than that of other hepatic malignant tumors, the 5-year survival rate is reported to be 64% after treatment. We present an unusual case of resectable HEHE of a single nodular type in the left lobe of the liver. The aim of the present study is to emphasize that multimodal treatment of radical resection followed by chemotherapy with thalidomide may achieve a relatively good survival outcome in patients with resectable HEHE of a single nodular type. We present an unusual case of resectable HEHE of the single nodular type in the left lobe of the liver in a patient who survived 15 years after multimodal treatment of radical resection and postoperative chemotherapy with thalidomide.
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