TY - JOUR
T1 - Overview of liposarcomas and their genomic landscape
AU - Keung, Emily Z.
AU - Somaiah, Neeta
N1 - Funding Information:
Dr. Keung is supported by National Institutes of Health (NIH) (T32 CA009599).
Publisher Copyright:
© The Author(s) 2019.
PY - 2019
Y1 - 2019
N2 - 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 inTP53,RB1, andNF1 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.
AB - 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 inTP53,RB1, andNF1 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.
KW - Dedifferentiated liposarcoma
KW - genetics
KW - genomics
KW - liposarcoma
KW - myxoid liposarcoma
KW - pleomorphic liposarcoma
KW - round cell liposarcoma
KW - well-differentiated liposarcoma
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U2 - 10.20517/jtgg.2019.03
DO - 10.20517/jtgg.2019.03
M3 - Review article
AN - SCOPUS:85113360737
SN - 2578-5281
VL - 3
JO - Journal of Translational Genetics and Genomics
JF - Journal of Translational Genetics and Genomics
M1 - 8
ER -