Retrospective Study
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jul 6, 2022; 10(19): 6437-6445
Published online Jul 6, 2022. doi: 10.12998/wjcc.v10.i19.6437
Neoadjuvant transcatheter arterial chemoembolization and systemic chemotherapy for the treatment of undifferentiated embryonal sarcoma of the liver in children
Min He, Jia-Bin Cai, Can Lai, Jun-Qing Mao, Jie-Ni Xiong, Zhong-Hai Guan, Lin-Jie Li, Qiang Shu, Mei-Dan Ying, Jin-Hu Wang
Min He, Jia-Bin Cai, Jun-Qing Mao, Jie-Ni Xiong, Zhong-Hai Guan, Lin-Jie Li, Qiang Shu, Jin-Hu Wang, Department of Surgical Oncology, The Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, Zhejiang Province, China
Can Lai, Department of Radiology, The Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, Zhejiang Province, China
Mei-Dan Ying, Cancer Center, Zhejiang University, Hangzhou 310058, Zhejiang Province, China
Author contributions: All authors contributed equally to this work; Wang JH was the guarantor and designed the study; He M drafted the initial manuscript; Wang JH and Ying MD helped to review the literature; Lai C and Wang JH conducted the TACE process; Mao JQ and Li LJ were the oncologists who treated the patient; Wang JH, He M, Cai JB, Xiong JN, Guan ZH and Shu Q were the surgeons who treated the patient; all authors issued final approval for the version to submitted.
Supported by Youth Program of Natural Science Foundation of Zhejiang Province, No. LQ20H160027; and National Natural Science Foundation of China, No. U20A20137.
Institutional review board statement: The study was reviewed and approved by the Children’s Hospital, Zhejiang University School of Medicine Institutional Review Board, No. 2021-IRB-252.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Jin-Hu Wang, MD, PhD, Chief Doctor, Director, Surgical Oncologist, Department of Surgical Oncology, The Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, No. 3333 Binsheng Road, Hangzhou 310052, Zhejiang Province, China. wjh@zju.edu.cn
Received: November 11, 2021
Peer-review started: November 11, 2021
First decision: March 3, 2022
Revised: March 23, 2022
Accepted: May 16, 2022
Article in press: May 16, 2022
Published online: July 6, 2022
Abstract
BACKGROUND

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare and aggressive mesenchymal tumor in children. Herein, we describe our experience in neoadjuvant therapy (NAT) and subsequent surgery for the treatment of UESL in children.

AIM

To evaluate the efficacy of NAT and explore a new choice for successful operation of UESL in children.

METHODS

We retrospectively analyzed six patients newly diagnosed with unresectable UESL who received NAT and then surgery at our center between January 2004 and December 2019. The tumor was considered unresectable if it involved a large part of both lobes of the liver or had invaded the main hepatic vessels or inferior vena cava. The NAT included preoperative transcatheter arterial chemoembolization (TACE) and systemic chemotherapy. The patients were 4 boys and 2 girls with a mean age of 7 years. The longest tumor at presentation ranged from 8.6 to 14.8 cm (mean, 12 cm). Extrahepatic metastases were present in 2 cases. Preoperative systemic chemotherapy was administered 3 wk after TACE. Tumor resection was performed 3 wk after one or two cycles of NAT. The patients received systemic chemotherapy after surgery.

RESULTS

All patients successfully underwent NAT and complete resection. The tumor volumes decreased by 18.2%–68.7%, with a mean decrease of 36% after 1 cycle of NAT (t = 3.524, P = 0.017). According to the Response Evaluation Criteria In Solid Tumors criteria, 4 patients had a partial response and underwent surgery, while 2 had stable disease and received another cycle of NAT before surgery. Massive tumor necrosis was seen on pathological examination of the surgical specimen: > 90% necrosis in two, > 50% necrosis in three, and 25% necrosis in 1, with an average of 71.8%. Post-NAT complications included fever, nausea and vomiting, and mild bone marrow suppression. Elevated alanine transaminase levels occurred in all patients, which returned to normal within 7–10 d after treatment. No cardiac or renal toxicity, severe hepatic dysfunction, bleeding and non-target embolization were observed in the patients. The median follow-up period was 8 years with an overall survival of 100%.

CONCLUSION

NAT effectively reduced tumor volume, cleared the tumor margin, and caused massive tumor necrosis. This may be a promising choice for successful surgery of UESL in children.

Keywords: Undifferentiated embryonal sarcoma of the liver, Unresectable, Neoadjuvant therapy, Transcatheter arterial chemoembolization, Systemic chemotherapy

Core Tip: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare and aggressive mesenchymal tumor in children. The prognosis of UESL has previously been considered poor. Modern multimodal treatment and supportive therapy have improved survival. Complete tumor excision plays the central role in the treatment of UESL. In this study, we analyzed our single-center data of children with UESL who received neoadjuvant therapy (NAT) between January 2004 and December 2019, and found that NAT effectively reduced tumor volume, cleared tumor margin, and caused massive tumor necrosis. It may provide a promising choice for successful operation of UESL in children.