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World J Gastrointest Oncol. Mar 15, 2020; 12(3): 248-266
Published online Mar 15, 2020. doi: 10.4251/wjgo.v12.i3.248
Hepatic Hemangioendothelioma: An update
Mayur Virarkar, Mohammed Saleh, Radwan Diab, Melissa Taggart, Peeyush Bhargava, Priya Bhosale
Mayur Virarkar, Mohammed Saleh, Radwan Diab, Priya Bhosale, Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
Melissa Taggart, Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
Peeyush Bhargava, Department of Radiology, The University of Medical Branch, Galveston, TX 77555, United States
Author contributions: All authors equally contributed to this paper with conception and design of the study, literature review and analysis, drafting, critical revision, editing, and approval of the final version.
Conflict-of-interest statement: Authors declare no conflict of interests for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Mayur Virarkar, MD, Doctor, Department of Diagnostic Radiology, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030, United States. mkvirarkar@mdanderson.org
Received: December 10, 2019
Peer-review started: April 26, 2020
First decision: May 24, 2020
Revised: February 21, 2020
Accepted: February 23, 2020
Article in press: February 23, 2020
Published online: March 15, 2020
Processing time: 93 Days and 7.9 Hours
Abstract

Primary epithelioid hemangioendotheliomas of the liver (EHL) are rare tumors with a low incidence. The molecular background of EHL is still under investigation, with WWTR1-CAMPTA1 mutation may function as a tumor marker. Commonly, this tumor is misdiagnosed with angiosarcoma, cholangiocarcinomas, metastatic carcinoma, and hepatocellular carcinoma (sclerosing variant). Characteristic features on imaging modalities such as ultrasound, computed tomography, magnetic resonance imaging and positron emission tomography/computed tomography guide in diagnosis and staging. The “halo sign” and the “lollipop sign” on computed tomography and magnetic resonance imaging are described in the literature. Currently, there are no standardized guidelines for treating EHL with treatment options are broad including: chemotherapy, ablation, surgery and liver transplantation with inconsistent results.

Keywords: Epithelioid hemangioendotheliomas; Halo sign; Lollipop sign; Angiosarcoma; Cholangiocarcinomas; Hepatocellular carcinoma

Core tip: Primary epithelioid hemangioendotheliomas of the liver are rare tumors with an incidence rate of less than 0.1 per 100000 population. The molecular background of epithelioid hemangioendotheliomas is still under investigation. The “halo sign” and the “lollipop sign” on computed tomography and magnetic resonance imaging are described in the literature. The differential diagnosis includes angiosarcoma, cholangiocarcinomas, metastatic carcinoma, and hepatocellular carcinoma (sclerosing variant). Currently, there are no standardized guidelines for treating EHL with treatment options are broad and include: chemotherapy, ablation, surgery and liver transplantation with inconsistent results.