Biology of EWS/FLI and Related Fusion Genesin Ewing’s Sarcoma and Primitive Neuroectodermal Tumor
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.
KeywordsPrimitive Neuroectodermal Tumor Desmoplastic Small Round Cell Tumor Transcriptional Activation Domain Small Round Cell Tumor Representational Difference Analysis
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- Downing JR, Head DR, Parham DM, Douglass EC, Hulshof MG, Link MP, Motroni TA, Grier HE, Curcio-Brint AM and Shapiro DN (1993) Detection of the (11;22)(q24;q12) translocation of Ewing’s peripheral neuroectodermal tumor by reverse transcription chain reaction. Am J Pathol 143: 1294 – 1300PubMedGoogle Scholar
- May WA, Gishizky ML, Lessnick SL, Lunsford LB, Lewis BC, Delattre O, Zucman J, Thomas G and Denny CT (1993a) Ewing sarcoma 11;22 translocation produces a chimeric factor that requires the DNA-binding domain encoded by FLU for transformation. Proc Natl Acad Sci USA 90: 5752 – 5756CrossRefGoogle Scholar
- May WA, Lessnick SL, Braun BS, Klemsz M, Lewis BC, Lunsford LB, Hromas R and Denny CT (1993b) The Ewing’s sarcoma EWS/FLI-1 fusion gene encodes a more transcriptional activator and is a more powerful transforming FLI-1. Mol Cell Biol 13: 7393 – 7398Google Scholar
- Triche TJ, Askin FB, Kissane JM (1987) Neuroblastoma, Ewing’s sarcoma, and the differential diagnosis of small-, round-, blue-cell tumors. In: Feingold M, Benningtion JC (eds) Major problems in pathology, vol 18. Saunders, Philadelphia, pp 145 – 195Google Scholar