Retrospective Study
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 26, 2020; 8(20): 4763-4772
Published online Oct 26, 2020. doi: 10.12998/wjcc.v8.i20.4763
Undifferentiated embryonal sarcoma of the liver: Clinical characteristics and outcomes
Chong Zhang, Chang-Jun Jia, Can Xu, Qiu-Ju Sheng, Xiao-Guang Dou, Yang Ding
Chong Zhang, Qiu-Ju Sheng, Xiao-Guang Dou, Yang Ding, Department of Infectious Diseases, Shengjing Hospital of China Medical University, Shenyang 110022, Liaoning Province, China
Chang-Jun Jia, Department of General Surgery, Shengjing Hospital of China Medical University, Shenyang 110022, Liaoning Province, China
Can Xu, Department of Pathology, Shengjing Hospital of China Medical University, Shenyang 110022, Liaoning Province, China
Author contributions: Zhang C and Ding Y designed and performed the research and wrote the paper; Dou XG designed the research and supervised the report; Sheng QJ contributed to the analysis; Jia CJ and Xu C provided clinical advice; all authors have read and approved the final manuscript.
Supported by The National Science and Technology Major Project, No. 2017ZX10201201 and No. 2017ZX10202202; and Liaoning Provincial Natural Science Foundation, No. 20180550096.
Institutional review board statement: The study was reviewed and approved by Shengjing Hospital of China Medical University Institutional Review Board (No. 2016PS248K).
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: The authors have no conflict of interest to declare.
Data sharing statement: No additional data are available.
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: Yang Ding, PhD, Professor, Department of Infectious Diseases, Shengjing Hospital of China Medical University, No. 39 Huaxiang Road, Tiexi District, Shenyang 110022, Liaoning Province, China. yding0903@sina.com
Received: June 28, 2020
Peer-review started: June 28, 2020
First decision: August 8, 2020
Revised: August 20, 2020
Accepted: August 29, 2020
Article in press: August 29, 2020
Published online: October 26, 2020
Processing time: 120 Days and 0.4 Hours
Abstract
BACKGROUND

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare liver malignancy originating from primary mesenchymal tissue. The clinical manifestations, laboratory tests, and imaging examinations of the disease lack specificity and the preoperative misdiagnosis rate is high. The overall prognosis is poor and survival rate is low.

AIM

To investigate the diagnosis, treatment, and prognosis of UESL.

METHODS

We performed a retrospective, single-center cohort study in Shengjing Hospital of China Medical University, which is a central hospital in northeast China. From 2005 to 2017, we recruited 14 patients with pathologically confirmed UESL. We analyzed the clinical manifestations, laboratory tests, imaging examinations, pathological examinations, therapy, and prognosis of these patients.

RESULTS

There were nine males and five females aged 2-60 years old included in the study. The major initial symptoms were abdominal pain (71.43%) and fever (57.14%). Preoperative laboratory tests revealed that seven patients had increased leukocyte levels, four showed a decrease in hemoglobin levels, seven patients had increased glutamyl transpeptidase levels, nine had increased lactate dehydrogenase levels, and three showed an increase in carbohydrate antigen 199. There was no difference in the rate of misdiagnosis in preoperative imaging examinations of UESL between adults and children (6/6 vs 5/8, P = 0.091). The survival rate after complete resection was 6/10, while that after incomplete resection was 0/4 (P = 0.040), suggesting that complete resection is important to improve survival rate. In total, five out of the eight children achieved survival. During the follow-up, the maximum survival time was shown to be 11 years and minimum survival time was 6 mo. Six adult patients relapsed late after surgery and all of them died.

CONCLUSION

Preoperative imaging examination for UESL has a high misdiagnosis rate. Multidisciplinary collaboration can improve the diagnostic accuracy of UESL. Complete surgical resection is the first choice for treatment of UESL.

Keywords: Undifferentiated embryonal sarcoma of the liver; Retrospective study; Clinical characteristics; Diagnosis; Treatment; Prognosis

Core Tip: Undifferentiated embryonal sarcoma of the liver is rarely seen in clinical practice; it is difficult to diagnose and easily misdiagnosed before surgery. Comprehensive treatment based on complete surgical resection is the key for long-term survival. The methods for diagnosis, treatment, and prognosis evaluation of the disease should be further investigated.