Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 26, 2022; 10(18): 6119-6127
Published online Jun 26, 2022. doi: 10.12998/wjcc.v10.i18.6119
Hepatic epithelioid hemangioendothelioma after thirteen years’ follow-up: A case report and review of literature
Wei-Fang Mo, Yu-Ling Tong
Wei-Fang Mo, Yu-Ling Tong, Department of General Practice, The 2nd Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou 310009, Zhejiang Province,China
Author contributions: Mo WF collected the data and wrote the manuscript; Tong YL designed the report; all authors have read and approve the final manuscript.
Supported by Zhejiang Medical and Health Science and Technology Project, No. 2021429795; and Scientific Research Project of Zhejiang Education Department, No. Y202043306.
Informed consent statement: Consent was obtained from the patient, who signed a written informed consent for publication of this case report.
Conflict-of-interest statement: The authors declare that they have no competing interests.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
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: Yu-Ling Tong, Doctor, Assistant Professor, Department of General Practice, The 2nd Affiliated Hospital of Zhejiang University, School of Medicine, No. 88 Jiefang Road, Hangzhou 310009, Zhejiang Province, China. tongyl0313@zju.edu.cn
Received: November 25, 2021
Peer-review started: November 25, 2021
First decision: January 12, 2022
Revised: January 24, 2022
Accepted: April 22, 2022
Article in press: April 22, 2022
Published online: June 26, 2022
Processing time: 203 Days and 12.2 Hours
Abstract
BACKGROUND

Hepatic epithelioid hemangioendothelioma (EHE) is a rare vascular endothelial cell tumor of the liver, consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix. Immunohistochemistry is usually positive for vascular markers, such as factor VIII-related antigen, CD31, and CD34. Hepatic EHE can have a varied clinical course; treatment includes liver transplantation, liver resection, chemotherapy, and radiation therapy.

CASE SUMMARY

A 46-year-old woman with abdominal discomfort and elevated serum carcinoembryonic antigen was found to have multiple low-density lesions in the liver and lung on computed tomography (CT) evaluation. An ultrasound-guided fine needle aspiration biopsy revealed a fibrous stroma with dendritic cells, containing intracellular vacuoles. Immunohistochemical staining found that the tumor cells were positive for CD34, CD31, and factor VIII-related antigen. The patient received four courses of combined chemotherapy and was followed-up for 13 years, at which time the patient was in stable condition without disease progression and a confined neoplasm, as evidenced by CT scans.

CONCLUSION

The histology and immunohistochemical characteristics of hepatic EHE are well described. Chemotherapy may be effective in patients with extrahepatic lesions.

Keywords: Epithelioid hemangioendothelioma, Liver neoplasm, Immunohistochemistry, Antineoplastic combined chemotherapy protocols, Treatment, Case report

Core Tip: The gold standard diagnosis for hepatic epithelioid hemangioendothelioma includes epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix, and positive vascular markers. Chemotherapy may be an effective treatment; close follow-up is necessary.