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Keung et al. J Transl Genet Genom 2019;3:8 Journal of Translational
DOI: 10.20517/jtgg.2019.03 Genetics and Genomics

Review Open Access

Overview of liposarcomas and their genomic
landscape

Emily Z. Keung , Neeta Somaiah 2
1
1 Department of Surgical Oncology, the University of Texas MD Anderson Cancer Center, Houston 77030, USA.
2 Department of Sarcoma Medical Oncology, the University of Texas MD Anderson Cancer Center, Houston 77030, USA.
Correspondence to: Dr. Neeta Somaiah, Department of Sarcoma Medical Oncology, the University of Texas MD Anderson Cancer
Center, Houston 77030, USA. E-mail: nsomaiah@mdanderson.org

How to cite this article: Keung EZ, Somaiah N. Overview of liposarcomas and their genomic landscape. J Transl Genet Genom 2019;3:8.
https://doi.org/10.20517/jtgg.2019.03
Received: 15 Feb 2019 First Decision: 5 March 2019 Revised: 11 Mar 2019 Accepted: 13 Mar 2019 Published: 22 May 2019

Science Editor: David Cooper Copy Editor: Cai-Hong Wang Production Editor: Huan-Liang Wu

Abstract
Liposarcoma (LPS) is among the most common soft tissue sarcoma affecting adults. LPS is divided into three biologic
subtypes characterized by specific genetic alterations. The most common LPS subtypes, well-differentiated and
dedifferentiated LPS, are nearly uniformly characterized by ring chromosomes and giant markers with chromosomal
amplification of 12q13-15 and resulting amplification of oncogenes MDM2, CDK4, and HMGA2. Myxoid/round cell LPS
commonly exhibits a distinctive (12; 16) translocation resulting in the FUS-DDIT3 fusion gene. Finally, pleomorphic LPS
harbors diverse complex genomic changes and chromosomal rearrangements and frequent mutations in TP53, RB1, and NF1
leading to dysregulation of tumor suppressor pathways. In this review, we summarize the currently available knowledge on
the genomics and genetics of LPS subtypes as well as recent advances in the multimodality management of LPS.

Keywords: Dedifferentiated liposarcoma, liposarcoma, genetics, genomics, myxoid liposarcoma, pleomorphic
liposarcoma, round cell liposarcoma, well-differentiated liposarcoma

INTRODUCTION
Soft tissue sarcomas (STS) encompass over 50 recognized entities according to the World Health
Organization (WHO) classification. Liposarcomas (LPS) are among the most common STS histologies,
[1]
representing 50% of retroperitoneal and 25% of extremity STS LPS consist of 3 biologic subgroups
encompassing 5 histologic subtypes characterized by specific genetic alterations [Table 1]. These three STS
subgroups, their characteristic genetic alterations, and treatment will be reviewed herein.

© The Author(s) 2019. Open Access This article is licensed under a Creative Commons Attribution 4.0
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