Surgical treatment of primary hepatic epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma is a very rare tumor of vascular origin. It can develop in different tissues such as soft tissue, lung, or liver. Hepatic epithelioid hemangioendothelioma (HEH) mostly affects females. The malignant potential of HEH often remains unclear in the individual patient. It can range from benign hemangioma to malignant hemangioendotheliosarcoma. Here we present our experience with five patients with primary HEH, who were treated with curative intention in our department. All patients in our series with confirmed histological HEH did not show extrahepatic extension and consequently underwent surgical treatment. In three patients, liver transplantation (LTx) was performed (two cadaveric and one living related). In one patient, a right-sided hemihepatectomy with partial resection of the diaphragm was performed. One patient died while she was on the waiting list for LTx due to rapid tumor progression. Postoperative follow-up ranged from 1 to 13 years. No adjuvant chemotherapy was applied. Until now, no recurrence of local tumor or distant metastases could be observed during follow-up in our series. Early detection and surgical intervention in case of HEH can potentially offer curative treatment. The treatment of first choice appears to be radical liver resection. In our view, LTx represents a potentially important option for patients with a nonresectable tumor. Despite the long waiting time, its often unclear dignity, and a proven progressive growth pattern, living related LTx also plays a potentially important role. The 5-year overall survival rate of patients with HEH in the literature varies from 43% to 55%. Long-term survival of patients with HEH is significantly higher compared to other hepatic malignancies. The role of adjuvant therapy currently remains unclear.