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.