Journal Title: بهار 86
Article title: Primary Malignant Hepatic Epithelioid Hemangioendothelioma، A Comprehensive Review of the Literature with Emphasis on the Surgical Therapy

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Article abstract:
Introduction & Objective: Malignant hepatic epithelioid hemangioendothelioma (HER) is a rare malignant tumor of vascular origin with unknown etiology and a variable natural course. The authors present a comprehensive review of the literature on HER with a focus on clinical outcome after different therapeutic strategies. Materials & Methods: All published series on patients with HEH (n = 434 patients) were: analyzed from the first description in 1984 to the current literature. The reviewed parameters included demographic data 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. The most common clinical manifestations were right upper quadrant pain, hepatomegaly, and weight loss. Most patients 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 most common 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% of patients). 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 been proposed as the treatment of choice because of the hepatic muIticentricity of HEH. In addition, LTx is an acceptable option for patients, who have HEH with extrahepatic manifestation. Highly selected patients may be able to undergo living-donor LTx, preserving the donor pool. The role of different adjuvant therapies for patients with HEH remains to be determined.
Article KeyWords:
Hepatic Epithelioid Hemangioendothelioma, Liver Transplantation, Liver Resection, Treatment
Article Authors:

	دكتر آريانب محـرابي	First Author	arianeb_mehrabi@med.uni-heidelberg.de
	دكتر آرش كشفـي	Other Author
	دكتر حميدرضا فنـوني	Other Author
	Peter Schemmer	Other Author
	Bruno M. Schmied	Other Author
	Peter Hallscheidt	Other Author
	Peter Schirmacher	Other Author