Biology of EWS/FLI and Related Fusion Genesin Ewing’s Sarcoma and Primitive Neuroectodermal Tumor

  • W. A. May
  • C. T. Denny
Part of the Current Topics in Microbiology and Immunology book series (CT MICROBIOLOGY, volume 220)


Ewing’s sarcoma and peripheral primitive neuroectodermal tumor (ES/PNET) are two related tumors of bone and soft tissue. Both malignancies belong to the enigmatic diagnostic category of small round cell tumors of childhood, which as a group occurs at a rate of 29 per million children (Triche et al. 1987). The precise cell of origin of ES/PNET is unknown. Circumstantial evidence suggests that these tumors may arise from neuroectodermal precursors (for review see Cavazzana et al. 1992). For example, ES/PNET cells express high levels of choline acetyl transferase, an enzyme important in the biosynthesis of cholinergic neurotransmitters. In addition, some ES/PNET cell lines can be induced in culture to form neurite extensions and express neurofilament proteins.


Primitive Neuroectodermal Tumor Desmoplastic Small Round Cell Tumor Transcriptional Activation Domain Small Round Cell Tumor Representational Difference Analysis 
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Copyright information

© Springer-Verlag Berlin Heidelberg 1997

Authors and Affiliations

  • W. A. May
    • 1
  • C. T. Denny
    • 1
    • 2
  1. 1.Department of Pediatrics, Gwynne Hazen Cherry Memorial Laboratories, Division of Hematology/Oncology, and Jonsson Comprehensive Cancer Center, School of MedicineUniversity of CaliforniaLos AngelesUSA
  2. 2.Department of Pediatrics, A2-312 MDCCUCLA Medical CenterLos AngelesUSA

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