PMID- 36059611
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220907
IS  - 2234-943X (Print)
IS  - 2234-943X (Electronic)
IS  - 2234-943X (Linking)
VI  - 12
DP  - 2022
TI  - Primary intrathoracic liposarcomas: A clinicopathologic and molecular study of 43 
      cases in one of the largest medical centers of China.
PG  - 949962
LID - 10.3389/fonc.2022.949962 [doi]
LID - 949962
AB  - INTRODUCTION: Primary intrathoracic liposarcoma is extremely rare, and most 
      published series lack genetic analyses. The aim of our study is to better 
      understand the clinicopathologic and genetic features of these rare lesions. 
      MATERIALS AND METHODS: Forty-three primary intrathoracic liposarcomas were 
      identified and most cases were analyzed by systematic genetic studies, including 
      fluorescence in situ hybridization (FISH), whole-exome sequencing (WES), and 
      Sanger sequencing. RESULTS: This series included 27 males and 16 females (ratios, 
      1.68:1) aged 24-73 years (median, 53 years). Tumors mainly occurred in the 
      mediastinum (n=23, 53.5%), followed by pleural cavity (n=16, 37.2%) and lung 
      (n=4, 9.3%). The study included 21 well-differentiated liposarcomas (WDLs), 19 
      dedifferentiated liposarcomas (DDLs), 2 myxoid pleomorphic liposarcomas (MPLs) 
      and 1 pleomorphic liposarcoma (PL), without identification of myxoid liposarcoma. 
      FISH analysis identified MDM2 amplification in 17 of 18 WDLs (94.4%) and all DDLs 
      (16/16, 100.0%). The MDM2-nonamplified WDL was CDK4-nonamplified but 
      FRS2-amplified. WES and Sanger sequencing found somatic TP53 mutation in the 2 
      MPLs. Follow-up information was available for 33 of 38 cases (86.8%). Thirteen 
      patients (39.4%) showed no evidence of disease, 10 patients (30.3%) were alive 
      with disease, and 8 patients (24.2%) died of disease. Fourteen cases developed 
      recurrence and 1 with metastasis. CONCLUSIONS: WDL/DDL was the overwhelming 
      subtype in this location, followed by MPL and PL. Analysis of the FRS2 gene, in 
      combination with MDM2 and other genes of 12q13-15, may more precisely 
      characterize WDL/DDLs. MPL is the most fatal subtype of this site. Further 
      studies are needed to explore the role of TP53 in the pathogenesis of MPL.
CI  - Copyright (c) 2022 Xie, Jing, Zhao, Peng, Chen, Lan, Peng, He, Chen, Zhang and 
      Zhang.
FAU - Xie, You
AU  - Xie Y
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
FAU - Jing, Wenyi
AU  - Jing W
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
FAU - Zhao, Wei
AU  - Zhao W
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
FAU - Peng, Ran
AU  - Peng R
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
FAU - Chen, Min
AU  - Chen M
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
FAU - Lan, Ting
AU  - Lan T
AD  - Department of Pathology, Sichuan Cancer Hospital and Institute, Sichuan Cancer 
      Center, Cancer Hospital Affiliate to School of Medicine, University of Electronic 
      Science and Technology of China, Chengdu, China.
FAU - Peng, Heng
AU  - Peng H
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
FAU - He, Xin
AU  - He X
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
FAU - Chen, Huijiao
AU  - Chen H
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
FAU - Zhang, Zhang
AU  - Zhang Z
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
FAU - Zhang, Hongying
AU  - Zhang H
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
LA  - eng
PT  - Journal Article
DEP - 20220817
PL  - Switzerland
TA  - Front Oncol
JT  - Frontiers in oncology
JID - 101568867
PMC - PMC9432863
OTO - NOTNLM
OT  - dedifferentiated liposarcoma
OT  - liposarcoma
OT  - molecular analysis
OT  - myxoid pleomorphic liposarcoma
OT  - thorax
OT  - well-differentiated liposarcoma
COIS- The authors declare that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2022/09/06 06:00
MHDA- 2022/09/06 06:01
PMCR- 2022/01/01
CRDT- 2022/09/05 03:40
PHST- 2022/05/22 00:00 [received]
PHST- 2022/07/27 00:00 [accepted]
PHST- 2022/09/05 03:40 [entrez]
PHST- 2022/09/06 06:00 [pubmed]
PHST- 2022/09/06 06:01 [medline]
PHST- 2022/01/01 00:00 [pmc-release]
AID - 10.3389/fonc.2022.949962 [doi]
PST - epublish
SO  - Front Oncol. 2022 Aug 17;12:949962. doi: 10.3389/fonc.2022.949962. eCollection 
      2022.