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Calinescu et al. Hepatoma Res 2021;7:54 Hepatoma Research
DOI: 10.20517/2394-5079.2021.25

Perspective Open Access

Surgical perspective on treatment of pediatric

undifferentiated sarcoma of the liver

1
1
Ana M. Calinescu , Barbara E. Wildhaber , Florent Guérin 2
1
Division of Pediatric Surgery, University Center of Pediatric Surgery of Western Switzerland, Geneva University Hospitals,
Geneva 1205, Switzerland.
2
Pediatric Surgery Unit, Bicêtre Hospital, Université Paris-Saclay, Paris 94275, France.
Correspondence to: Dr. Ana M. Calinescu, Division of Pediatric Surgery, University Center of Pediatric Surgery of Western
Switzerland, Geneva University Hospitals, 6 Rue Willy Donze, Geneva 1205, Switzerland. E-mail: ana-maria.calinescu@hcuge.ch
How to cite this article: Calinescu AM, Wildhaber BE, Guérin F. Surgical perspective on treatment of pediatric undifferentiated
sarcoma of the liver. Hepatoma Res 2021;7:54. https://dx.doi.org/10.20517/2394-5079.2021.25
Received: 27 Feb 2021 First Decision: 12 Apr 2021 Revised: 13 May 2021 Accepted: 25 May 2021 First online: 31 May 2021

Academic Editors: Guang-Wen Cao, Piotr Czauderna Copy Editor: Yue-Yue Zhang Production Editor: Yue-Yue Zhang

Abstract
Surgical resection and chemotherapy are the mainstay of the treatment for undifferentiated embryonal sarcoma of
the liver. Whether neoadjuvant chemotherapy should be systematically performed is a matter of debate;
perioperative morbidity and mortality should be carefully weighed against chemotherapy-associated
complications. In order to manage undifferentiated embryonal sarcoma of the liver and to allow for accurate
outcome analysis, there is a clear need for standardization of disease extent as well as for a risk stratification
system, including the PRETEXT grouping system, patient age, and tumor size.

Keywords: Undifferentiated embryonal sarcoma of the liver, staging system, positive margins, neoadjuvant
chemotherapy

INTRODUCTION
Undifferentiated embryonal sarcoma of the liver (UESL) is the third most frequent liver malignancy in
children. Its peak of incidence lays between that of hepatoblastoma (young children) and hepatocellular
carcinoma (older children) . Most children who develop UESL are aged 6 to 10 years . The clinical
[1]
[2]
presentation is often non-specific: an abdominal mass with or without abdominal pain can be observed,
fever if hemorrhage, and/or necrosis, as well as secondary symptoms (e.g., weight loss, anorexia, etc.) .
[3]

© The Author(s) 2021. Open Access This article is licensed under a Creative Commons Attribution 4.0
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