PMID- 10571517
OWN - NLM
STAT- MEDLINE
DCOM- 19991130
LR  - 20190722
IS  - 0046-8177 (Print)
IS  - 0046-8177 (Linking)
VI  - 30
IP  - 11
DP  - 1999 Nov
TI  - Absence of EWS/FLI1 fusion in olfactory neuroblastomas indicates these tumors do 
      not belong to the Ewing's sarcoma family.
PG  - 1356-60
AB  - The balanced reciprocal translocation t(11;22)(q24;q12) is a specific molecular 
      marker for the Ewing's sarcoma family of tumors (ESFT). Based on the detection of 
      this translocation in some olfactory neuroblastomas (ONBs), it has been proposed 
      that ONBs also belong to the ESFT. Others have challenged this hypothesis; 
      however, the rarity of ONBs, as well as the lack of molecular techniques that 
      work reliably on formalin fixed tissue, have precluded the molecular analysis of 
      a significant number of cases. We evaluated the immunophenotypic and molecular 
      features of 17 paranasal small round neuroectodermal tumors using routinely fixed 
      tissue. Probes localizing to 22q12 (EWS) and 11q24 (FLI-1) were used in a 
      standard 2-color fluorescence in situ hybridization (FISH) assay to evaluate 
      EWS/FLI1 fusion on the der(22). Sixteen tumors were mic-2 negative, whereas 1 
      tumor was mic-2 positive, compatible with ONB and ESFT, respectively. Thirteen of 
      15 ONBs could be evaluated by FISH, and all 13 were negative for the EWS/FLI1 
      fusion. Distinct fusion signals were identified in the single paranasal ESFT. Our 
      findings indicate that ONBs lack the EWS/FLI1 fusion and are unrelated to the 
      ESFT; however, true ESFTs may rarely occur as primary sinonasal tumors.
FAU - Kumar, S
AU  - Kumar S
AD  - Laboratory of Pathology, National Cancer Institute, and Cancer Genetics Branch, 
      National Human Genome Research Institute, National Institutes of Health, 
      Bethesda, MD 20892-1500, USA.
FAU - Perlman, E
AU  - Perlman E
FAU - Pack, S
AU  - Pack S
FAU - Davis, M
AU  - Davis M
FAU - Zhang, H
AU  - Zhang H
FAU - Meltzer, P
AU  - Meltzer P
FAU - Tsokos, M
AU  - Tsokos M
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Hum Pathol
JT  - Human pathology
JID - 9421547
RN  - 0 (EWS-FLI fusion protein)
RN  - 0 (Oncogene Proteins, Fusion)
RN  - 0 (Proto-Oncogene Protein c-fli-1)
RN  - 0 (RNA-Binding Protein EWS)
RN  - 0 (Transcription Factors)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Child
MH  - Child, Preschool
MH  - Diagnosis, Differential
MH  - Esthesioneuroblastoma, Olfactory/*metabolism
MH  - Female
MH  - Humans
MH  - Immunohistochemistry
MH  - In Situ Hybridization, Fluorescence
MH  - Male
MH  - Middle Aged
MH  - *Nasal Cavity
MH  - Nose Neoplasms/*metabolism
MH  - Oncogene Proteins, Fusion/*metabolism
MH  - Proto-Oncogene Protein c-fli-1
MH  - RNA-Binding Protein EWS
MH  - Transcription Factors/*metabolism
EDAT- 1999/11/26 00:00
MHDA- 1999/11/26 00:01
CRDT- 1999/11/26 00:00
PHST- 1999/11/26 00:00 [pubmed]
PHST- 1999/11/26 00:01 [medline]
PHST- 1999/11/26 00:00 [entrez]
AID - S0046-8177(99)90068-0 [pii]
AID - 10.1016/s0046-8177(99)90068-0 [doi]
PST - ppublish
SO  - Hum Pathol. 1999 Nov;30(11):1356-60. doi: 10.1016/s0046-8177(99)90068-0.