PMID- 19737655
OWN - NLM
STAT- MEDLINE
DCOM- 20090925
LR  - 20171116
IS  - 1873-4456 (Electronic)
IS  - 0165-4608 (Linking)
VI  - 194
IP  - 1
DP  - 2009 Oct
TI  - Ewing sarcoma-peripheral neuroectodermal tumor of the kidney with a FUS-ERG 
      fusion transcript.
PG  - 53-7
LID - 10.1016/j.cancergencyto.2009.06.002 [doi]
AB  - The Ewing family of tumors (EFTs) represents a neoplastic entity characterized by 
      specific chromosomal rearrangements. The most commonly detected translocation 
      involves fusion of EWSR1 to one of the genes encoding ETS family of transcription 
      factors, usually FLI1 or ERG. In rare cases, FUS or FEV has been shown to 
      substitute for EWSR1. The detection of specific translocations by fluorescence in 
      situ hybridization (FISH), reverse transcription-polymerase chain reaction 
      (RT-PCR), or both has become a diagnostic hallmark for the EFTs. We report here a 
      case of small round blue cell tumor detected in the kidney of a 3-year-old girl. 
      The use of FISH in combination with RT-PCR led to identification of a novel 
      FUS-ERG fusion transcript in the tumor, with FUS exons 1-5 fused in-frame to ERG 
      exons 6-9. The results from the molecular analyses were critical for reaching a 
      final diagnostic conclusion of Ewing tumor-peripheral neuroectodermal tumor and 
      hence had important therapeutic implications for the patient.
FAU - Berg, Thomas
AU  - Berg T
AD  - Department of Pathology, University Hospital of Northern Norway, Tromso, Norway. 
      Thomas.Berg@unn.no
FAU - Kalsaas, Ann-Hilde
AU  - Kalsaas AH
FAU - Buechner, Jochen
AU  - Buechner J
FAU - Busund, Lill-Tove
AU  - Busund LT
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Cancer Genet Cytogenet
JT  - Cancer genetics and cytogenetics
JID - 7909240
RN  - 0 (12E7 Antigen)
RN  - 0 (Antigens, CD)
RN  - 0 (CD99 protein, human)
RN  - 0 (Cell Adhesion Molecules)
RN  - 0 (Oncogene Proteins, Fusion)
RN  - 0 (RNA-Binding Protein FUS)
RN  - 0 (S100 Proteins)
RN  - 0 (TLS-ERG fusion protein, human)
RN  - 0 (Vimentin)
SB  - IM
MH  - 12E7 Antigen
MH  - Antigens, CD/analysis
MH  - Cell Adhesion Molecules/analysis
MH  - Child, Preschool
MH  - Diagnosis, Differential
MH  - Female
MH  - Humans
MH  - Immunohistochemistry
MH  - In Situ Hybridization, Fluorescence
MH  - Kidney/metabolism/pathology
MH  - Kidney Neoplasms/*diagnosis/genetics/metabolism
MH  - Neuroectodermal Tumors, Primitive, Peripheral/*diagnosis/genetics/metabolism
MH  - Oncogene Proteins, Fusion/*genetics
MH  - RNA-Binding Protein FUS/*genetics
MH  - Reverse Transcriptase Polymerase Chain Reaction
MH  - S100 Proteins/analysis
MH  - Sarcoma, Ewing/*diagnosis/genetics/metabolism
MH  - Vimentin/analysis
EDAT- 2009/09/10 06:00
MHDA- 2009/09/26 06:00
CRDT- 2009/09/10 06:00
PHST- 2009/05/06 00:00 [received]
PHST- 2009/06/02 00:00 [revised]
PHST- 2009/06/05 00:00 [accepted]
PHST- 2009/09/10 06:00 [entrez]
PHST- 2009/09/10 06:00 [pubmed]
PHST- 2009/09/26 06:00 [medline]
AID - S0165-4608(09)00293-3 [pii]
AID - 10.1016/j.cancergencyto.2009.06.002 [doi]
PST - ppublish
SO  - Cancer Genet Cytogenet. 2009 Oct;194(1):53-7. doi: 
      10.1016/j.cancergencyto.2009.06.002.