Retrospective Study
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 16, 2022; 10(17): 5606-5619
Published online Jun 16, 2022. doi: 10.12998/wjcc.v10.i17.5606
Hepatic epithelioid hemangioendothelioma: Clinical characteristics, diagnosis, treatment, and prognosis
Man Zhao, Fei Yin
Man Zhao, Fei Yin, Department of Gastroenterology, The Fourth Hospital of Hebei Medical University, Shijiazhuang 050000, Hebei Province, China
Author contributions: Yin F designed the research study; Zhao M and Yin F performed the research, analyzed the data, and wrote the manuscript; all authors have read and approved the final manuscript.
Institutional review board statement: The study was reviewed and approved by the Ethics Committee and Institutional Review Board of The Fourth Hospital of Hebei Medical University.
Conflict-of-interest statement: There are no conflicts of interest to report.
Data sharing statement: The data that support the findings of this study are available from the corresponding author, upon reasonable request.
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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Fei Yin, PhD, Chief Physician, Department of Gastroenterology, The Fourth Hospital of Hebei Medical University, No. 12 Jiankang Road, Shijiazhuang 050000, Hebei Province. China. yinfei_4y@sina.com
Received: December 1, 2021
Peer-review started: December 1, 2021
First decision: January 12, 2022
Revised: January 23, 2022
Accepted: April 15, 2022
Article in press: April 15, 2022
Published online: June 16, 2022
ARTICLE HIGHLIGHTS
Research background

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare hepatic vascular tumor with unpredictable malignant potential.

Research motivation

The etiology, characteristics, diagnosis, treatment, and prognosis of HEHE are not well-understood, and large-scale retrospective studies are required to better understand this disease.

Research objectives

To determine the characteristics of HEHE and identify its optimal treatments and prognostic factors.

Research methods

The clinical data of two patients diagnosed with HEHE at the Fourth Hospital of Hebei Medical University and 258 previously reported cases retrieved from the China National Knowledge Infrastructure and PubMed databases between 1996 and 2021 were combined and summarized. Information such as clinical features, laboratory examination findings, imaging findings, pathological characteristics, treatment, and survival periods were reviewed. Kaplan-Meir curves were used for survival analysis. Prognostic factors were identified by Cox regression analysis.

Research results

The management options for patients with HEHE included liver resection (LR, 29.7%), liver transplantation (16.1%), palliative treatments (12.7%), transhepatic arterial chemotherapy and embolization (10.2%), chemotherapy (11.0%), antiangiogenic therapy (15.3%), and other treatments (5.1%); the mean survival time was 158.6, 147.3, 4.2, 90.8, 71.4, 83.1, and 55.0 mo, respectively. Multivariate analysis showed that liver function (P = 0.045), intrahepatic metastasis (P = 0.029), and treatment (P = 0.045) were independent prognostic factors.

Research conclusions

The clinical course of HEHE is rare and variable, and patients with intrahepatic metastases and liver dysfunction may have a poorer prognosis than those without. Surgical intervention, whether LR or transplantation, might be warranted regardless of extrahepatic metastasis. For patients without the option for surgery, clinicians should consider the use of transhepatic arterial chemotherapy and embolization with antiangiogenic drugs in the treatment of HEHE.

Research perspectives

Large prospective studies are needed to determine the best nonsurgical treatment options.