PMID- 32039736
OWN - NLM
STAT- MEDLINE
DCOM- 20201125
LR  - 20240328
IS  - 1746-1596 (Electronic)
IS  - 1746-1596 (Linking)
VI  - 15
IP  - 1
DP  - 2020 Feb 10
TI  - Primary pulmonary myxoid sarcoma with EWSR1-CREB1 fusion: a case report and 
      review of the literature.
PG  - 15
LID - 10.1186/s13000-020-00930-2 [doi]
LID - 15
AB  - BACKGROUND: Primary pulmonary myxoid sarcoma (PPMS) is an extremely rare lung 
      sarcoma that is characterized in most cases by recurrent balanced chromosomal 
      translocation t(2;22)(q33;q12) leading to the oncogenic fusion gene EWSR1-CREB1. 
      CASE PRESENTATION: We report a case of PPMS with molecular confirmation using 
      fluorescence in situ hybridization (FISH) and DNA sequencing in a 45-year-old 
      female patient. Computer tomography (CT) scanning revealed a peripheral 
      circumscribed solid mass of 2.1 x 2 cm in the right lung superior lobe. 
      Histologically, the tumor cells ranged from stellate, polygonal to 
      chondrocyte-like or physaliferous-like, forming reticular network of delicate 
      lace-like cellular strands and cords in abundant myxoid stroma. The tumor cell 
      immunophenotype was positive for vimentin, EMA and negative for CK-pan, TTF-1, 
      CAM5.2, S-100, calponin, SMA, desmin, ALK, CD31 and CD34. Molecular analysis 
      demonstrated EWSR1-CREB1 gene fusion in this tumor. During 38 months of 
      follow-up, the patient was alive with no clinical or radiological evidence of 
      recurrence or metastasis. CONCLUSION: PPMS is a rare low-grade sarcoma with 
      distinct histological and genetic features. We add another case to the literature 
      of this rare tumor and report for the first time occurrence of chondrocyte-like 
      and physaliferous-like tumor cells in this tumor, thus enriching its morphologic 
      and cytologic spectrum.
FAU - Chen, Zhenwei
AU  - Chen Z
AD  - Department of Pathology, Jinhua Municipal Central Hospital, 351 Mingyue Road, 
      Jinhua, 321000, Zhejiang Province, People's Republic of China.
FAU - Yang, Yihui
AU  - Yang Y
AD  - Department of Pathology, Jinhua Municipal Central Hospital, 351 Mingyue Road, 
      Jinhua, 321000, Zhejiang Province, People's Republic of China.
FAU - Chen, Rongming
AU  - Chen R
AD  - Department of Pathology, the People's Hospital of Changfeng County, Changfeng 
      County, Anhui Province, People's Republic of China.
FAU - Ng, Chi Sing
AU  - Ng CS
AUID- ORCID: 0000-0002-0097-5479
AD  - Department of Pathology, St. Teresa's Hospital, Kowloon, Hong Kong. 
      ngcspeter@gmail.com.
FAU - Shi, Hongqi
AU  - Shi H
AD  - Department of Pathology, Jinhua Municipal Central Hospital, 351 Mingyue Road, 
      Jinhua, 321000, Zhejiang Province, People's Republic of China.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
DEP - 20200210
PL  - England
TA  - Diagn Pathol
JT  - Diagnostic pathology
JID - 101251558
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (CREB1 protein, human)
RN  - 0 (Cyclic AMP Response Element-Binding Protein)
RN  - 0 (EWSR1 protein, human)
RN  - 0 (RNA-Binding Protein EWS)
SB  - IM
MH  - Biomarkers, Tumor/analysis
MH  - Cyclic AMP Response Element-Binding Protein/*metabolism
MH  - Female
MH  - Humans
MH  - Middle Aged
MH  - Neoplasm Recurrence, Local/diagnosis/*metabolism/pathology
MH  - RNA-Binding Protein EWS/*metabolism
MH  - Sarcoma/diagnosis/*pathology
MH  - Soft Tissue Neoplasms/pathology
PMC - PMC7008573
OTO - NOTNLM
OT  - CREB1
OT  - Chondrocyte-like
OT  - EWSR1
OT  - Myxoid
OT  - Physaliferous-like
OT  - Pulmonary
OT  - Sarcoma
COIS- The authors declare that they have no competing interests.
EDAT- 2020/02/11 06:00
MHDA- 2020/11/26 06:00
PMCR- 2020/02/10
CRDT- 2020/02/11 06:00
PHST- 2019/08/18 00:00 [received]
PHST- 2020/02/05 00:00 [accepted]
PHST- 2020/02/11 06:00 [entrez]
PHST- 2020/02/11 06:00 [pubmed]
PHST- 2020/11/26 06:00 [medline]
PHST- 2020/02/10 00:00 [pmc-release]
AID - 10.1186/s13000-020-00930-2 [pii]
AID - 930 [pii]
AID - 10.1186/s13000-020-00930-2 [doi]
PST - epublish
SO  - Diagn Pathol. 2020 Feb 10;15(1):15. doi: 10.1186/s13000-020-00930-2.