Abstract
Ewing-like sarcomas are also known as Ewing-like sarcoma family, Ewing-like tumor family, atypical Ewing sarcomas, and Ewing-like tumors. The terms Ewing sarcoma family of tumors and Ewing family of tumors include both Ewing sarcoma (osseous and extraosseous) and Ewing-like sarcomas.
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Notes
- 1.
Round cell sarcomas are a heterogeneous group of mesenchymal neoplasms with overlapping morphology and immunohistochemical profile. Ewing sarcoma is the most well-known tumor in this group characterized by EWSR1/FUS rearrangements with members of the ETS family of transcription factors: undifferentiated round cell sarcomas lacking these rearrangements are known as Ewing-like sarcomas, described in this section.
- 2.
CIC-DUX4 fusion gene: the CIC gene is the human homolog of the Drosophila gene capicua transcriptional repressor; CIC encodes a high-mobility group box transcription factor and is involved in the development of the central nervous system. DUX4 is a double homeodomain gene whose normal function is unclear. The transcription activity of the CIC-DUX4 fusion product (which is linked to the CIC component) leads to the upregulation of the PEA3 subclass of ETS family of genes, which includes ETV1, ETV4, and ETV-5.
- 3.
BCOR-CCNB3 fusion gene: this fusion originates from a paracentric inversion on the X-chromosome and splicing of the end of the BCOR coding sequence to the CCNB3 exon 5 splice acceptor site. The resultant fusion protein is composed of full-length BCOR, a transcriptional repressor encoding the Bcl-6 co-repressor, and the C-terminus of cyclin CCNB3. The BCOR-CCNB3 fusion protein is oncogenic and is believed to drive cell proliferation in this sarcoma.
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Mocellin, S. (2021). Ewing-Like Sarcomas. In: Soft Tissue Tumors . Springer, Cham. https://doi.org/10.1007/978-3-030-58710-9_88
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DOI: https://doi.org/10.1007/978-3-030-58710-9_88
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