Primary Malignant Hepatic Epithelioid Hemangioendothelioma
نویسندگان
1 Department of General, Visceral, and Transplant Surgery, University of Heidelberg, Heidelberg, Germany
2 Department of Radiology, University of Heidelberg, Heidelberg, Germany
3 Department of Pathology, University of Heidelberg, Heidelberg, Germany
4 Department of General, Visceral, and Transplant Surgery, University of Heidelberg, Heidelberg, Germany
5 Department of General, Visceral, and Transplant Surgery, University of Heidelberg, Heidelberg, Germany
6 Department of General, Visceral, and Transplant Surgery, University of Heidelberg, Heidelberg, Germany
7 Department of General, Visceral, and Transplant Surgery, University of Heidelberg, Heidelberg, Germany
8 Department of General, Visceral, and Transplant Surgery, University of Heidelberg, Heidelberg, Germany
9 Department of General, Visceral, and Transplant Surgery, University of Heidelberg, Heidelberg, Germany
10 Department of General, Visceral, and Transplant Surgery, University of Heidelberg, Heidelberg, Germany
11 Department of General, Visceral, and Transplant Surgery, University of Heidelberg, Heidelberg, Germany
doi
چکیده
Introduction & Objective: Malignant hepatic epithelioid hemangioendothelioma (HER) is a raremalignant tumor of vascular origin with unknown etiology and a variable natural course. The authors present acomprehensive review of the literature on HER with a focus on clinical outcome after different therapeuticstrategies.Materials & Methods: All published series on patients with HEH (n = 434 patients) were: analyzedfrom the first description in 1984 to the current literature. The reviewed parameters included demographicdata clinical manifestations, therapeutic modalities, and clinical outcome.Results: The mean age of patients with HER was 41.7 years and the male-to-female ratio was 2:3. Themost common clinical manifestations were right upper quadrant pain, hepatomegaly, and weight loss. Mostpatients presented with multifocal tumor that involved both lobes of the liver. Lung peritoneum, lymph nodes,and bone were the most common sites of extrahepatic involvement at the time of diagnosis. The mostcommon management has been liver transplantation (LTx) (44.8% of patients), followed by no treatment(24.8% of patients), chemo-therapy or radiotherapy (21% of patients), and liver resection (LRx) (9.4% ofpatients). The 1-year and 5-year patient survival rates were 96% and 54.5%, respectively, after LTx; 39.3%and 4.5%, respectively; after no treatment, 73.3% and 30%, respectively, after chemotherapy or radiotherapy;and 100% and 75%, respectively, after LRx.Conclusions: LRx has been the treatment of chosen in patients with resectable HER. LTx has beenproposed as the treatment of choice because of the hepatic muIticentricity of HEH. In addition, LTx is anacceptable option for patients, who have HEH with extrahepatic manifestation. Highly selected patients maybe able to undergo living-donor LTx, preserving the donor pool. The role of different adjuvant therapies forpatients with HEH remains to be determined.
