PMID- 8577710
OWN - NLM
STAT- MEDLINE
DCOM- 19960314
LR  - 20190501
IS  - 0027-8424 (Print)
IS  - 1091-6490 (Electronic)
IS  - 0027-8424 (Linking)
VI  - 93
IP  - 3
DP  - 1996 Feb 6
TI  - Olfactory neuroblastoma is a peripheral primitive neuroectodermal tumor related 
      to Ewing sarcoma.
PG  - 1038-43
AB  - Olfactory neuroblastoma (ONB) is a malignant tumor of the nasal mucosa whose 
      histogenesis is unclear. A relationship to neuroblastoma (NB), a pediatric tumor 
      of the sympathetic nervous system, is based on morphologic similarities and the 
      expression of similar neural antigens. However, the clinical presentation of ONB 
      differs from that of NB, and MYCN amplification characteristic of NB is not 
      observed. We have therefore examined the relationship of this malignancy to other 
      classes of neural tumors. In previous studies, two ONB cell lines demonstrated 
      cytogenetic features and patterns of protooncogene expression suggestive of a 
      relationship to the Ewing sarcoma family of childhood peripheral primitive 
      neuroectodermal tumors (pPNETs). The pPNETs show t(11;22)(q24;q12) or 
      t(21;22)(q22;q12) chromosomal translocations fusing the EWS gene from 22q12 with 
      either the FL11 gene on 11q24 or the ERG gene on 21q22. We therefore analyzed 
      ONBs for the presence of pPNET-associated gene fusions. Both cell lines showed 
      rearrangement of the EWS gene, and fluorescence in situ hybridization (FISH) of 
      each case demonstrated fusion of EWS and FL11 genomic sequences. Moreover, both 
      lines expressed EWS/FL11 fusion transcripts with in-frame junctions between exon 
      7 of EWS and exon 6 of FL11 as described for pPNETs. We identified similar gene 
      fusions in four of six primary ONB cases. None of the cases expressed tyrosine 
      hydroxylase, a catecholamine biosynthetic enzyme widely expressed in NB. Our 
      studies indicate that ONB is not a NB but is a member of the pPNET family.
FAU - Sorensen, P H
AU  - Sorensen PH
AD  - Department of Pathology, British Columbia's Children's Hospital, Vancouver, 
      Canada.
FAU - Wu, J K
AU  - Wu JK
FAU - Berean, K W
AU  - Berean KW
FAU - Lim, J F
AU  - Lim JF
FAU - Donn, W
AU  - Donn W
FAU - Frierson, H F
AU  - Frierson HF
FAU - Reynolds, C P
AU  - Reynolds CP
FAU - Lopez-Terrada, D
AU  - Lopez-Terrada D
FAU - Triche, T J
AU  - Triche TJ
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Proc Natl Acad Sci U S A
JT  - Proceedings of the National Academy of Sciences of the United States of America
JID - 7505876
RN  - 0 (DNA Primers)
RN  - 0 (Heterogeneous-Nuclear Ribonucleoproteins)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (RNA-Binding Protein EWS)
RN  - 0 (Recombinant Fusion Proteins)
RN  - 0 (Ribonucleoproteins)
SB  - IM
MH  - Adult
MH  - Bone Neoplasms/classification/*genetics
MH  - Cell Line
MH  - Child
MH  - Chromosome Mapping
MH  - Chromosomes, Human, Pair 11
MH  - Chromosomes, Human, Pair 21
MH  - Chromosomes, Human, Pair 22
MH  - DNA Primers
MH  - Female
MH  - Gene Expression
MH  - Gene Rearrangement
MH  - Heterogeneous-Nuclear Ribonucleoproteins
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Male
MH  - Middle Aged
MH  - Nasal Mucosa
MH  - Neoplasm Proteins/genetics
MH  - Neuroblastoma/classification/*genetics
MH  - Neuroectodermal Tumors, Primitive/classification/*genetics
MH  - Nose Neoplasms/classification/*genetics
MH  - Polymerase Chain Reaction
MH  - RNA-Binding Protein EWS
MH  - Recombinant Fusion Proteins/biosynthesis
MH  - Ribonucleoproteins/biosynthesis/genetics
MH  - Sarcoma, Ewing/classification/*genetics
MH  - Translocation, Genetic
MH  - Tumor Cells, Cultured
PMC - PMC40026
EDAT- 1996/02/06 00:00
MHDA- 1996/02/06 00:01
PMCR- 1996/08/06
CRDT- 1996/02/06 00:00
PHST- 1996/02/06 00:00 [pubmed]
PHST- 1996/02/06 00:01 [medline]
PHST- 1996/02/06 00:00 [entrez]
PHST- 1996/08/06 00:00 [pmc-release]
AID - 10.1073/pnas.93.3.1038 [doi]
PST - ppublish
SO  - Proc Natl Acad Sci U S A. 1996 Feb 6;93(3):1038-43. doi: 10.1073/pnas.93.3.1038.