PMID- 15139001
OWN - NLM
STAT- MEDLINE
DCOM- 20040727
LR  - 20151119
IS  - 1045-2257 (Print)
IS  - 1045-2257 (Linking)
VI  - 40
IP  - 3
DP  - 2004 Jul
TI  - The chimeric FUS/CREB3l2 gene is specific for low-grade fibromyxoid sarcoma.
PG  - 218-28
AB  - Low-grade fibromyxoid sarcoma (LGFMS) is a variant of fibrosarcoma that was 
      recognized as a distinct tumor entity only quite recently. We previously 
      described a translocation, t(7;16)(q33;p11), that resulted in a fusion of the FUS 
      and CREB3L2 (also known as BBF2H7) genes in two soft tissue tumors that fulfilled 
      morphologic criteria for LGFMS. To delineate the spectrum of tumors that may 
      harbor the FUS/CREB3L2 gene, we selected 45 low-grade spindle cell sarcomas for 
      reverse transcriptase polymerase chain reaction (RT-PCR) and/or fluorescence in 
      situ hybridization (FISH) analyses; none of these tumors had originally been 
      diagnosed as LGFMS. Furthermore, also included were two benign soft tissue tumors 
      and nine high-grade sarcomas with supernumerary ring chromosomes or 7q3 
      rearrangement and three tumors diagnosed as LGFMS prior to the genetic analysis. 
      Of the 59 tumors analyzed, 12 were FUS/CREB3L2-positive, all of which were 
      diagnosed at histopathologic re-examination as being LGFMS, of both the classical 
      subtype and the subtype with giant collagen rosettes. The breakpoints in the 
      fusion transcripts were always in exons 6 or 7 of FUS and exon 5 of CREB3L2. The 
      results indicated that FUS/CREB3L2 is specifically associated with LGFMS and that 
      RT-PCR or FISH analysis may be useful for the differential diagnosis.
CI  - Copyright 2004 Wiley-Liss, Inc.
FAU - Panagopoulos, Ioannis
AU  - Panagopoulos I
AD  - Department of Clinical Genetics, Lund University Hospital, Lund, Sweden.
FAU - Storlazzi, Clelia Tiziana
AU  - Storlazzi CT
FAU - Fletcher, Christopher D M
AU  - Fletcher CD
FAU - Fletcher, Jonathan A
AU  - Fletcher JA
FAU - Nascimento, Antonio
AU  - Nascimento A
FAU - Domanski, Henryk A
AU  - Domanski HA
FAU - Wejde, Johan
AU  - Wejde J
FAU - Brosjo, Otte
AU  - Brosjo O
FAU - Rydholm, Anders
AU  - Rydholm A
FAU - Isaksson, Margareth
AU  - Isaksson M
FAU - Mandahl, Nils
AU  - Mandahl N
FAU - Mertens, Fredrik
AU  - Mertens F
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Genes Chromosomes Cancer
JT  - Genes, chromosomes & cancer
JID - 9007329
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (CREB3 protein, human)
RN  - 0 (Cyclic AMP Response Element-Binding Protein)
RN  - 0 (RNA-Binding Protein FUS)
RN  - 0 (Recombinant Fusion Proteins)
RN  - 0 (Transcription Factors)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Amino Acid Sequence/genetics
MH  - Base Sequence/genetics
MH  - *Biomarkers, Tumor/genetics
MH  - Chromosome Mapping/methods
MH  - Cyclic AMP Response Element-Binding Protein
MH  - Cytogenetic Analysis/methods
MH  - Female
MH  - Fibrosarcoma/classification/*diagnosis/genetics
MH  - Humans
MH  - In Situ Hybridization, Fluorescence/methods
MH  - Male
MH  - Middle Aged
MH  - Molecular Sequence Data
MH  - RNA-Binding Protein FUS/*genetics
MH  - *Recombinant Fusion Proteins/genetics
MH  - Reverse Transcriptase Polymerase Chain Reaction/methods
MH  - Sarcoma/diagnosis/genetics
MH  - Soft Tissue Neoplasms/diagnosis/genetics
MH  - Transcription Factors/*genetics
EDAT- 2004/05/13 05:00
MHDA- 2004/07/28 05:00
CRDT- 2004/05/13 05:00
PHST- 2004/05/13 05:00 [pubmed]
PHST- 2004/07/28 05:00 [medline]
PHST- 2004/05/13 05:00 [entrez]
AID - 10.1002/gcc.20037 [doi]
PST - ppublish
SO  - Genes Chromosomes Cancer. 2004 Jul;40(3):218-28. doi: 10.1002/gcc.20037.