Changes

<span style="color:#0070C0">(''General Instructions – The main focus of these pages is the clinically significant genetic alterations in each disease type. Use [https://www.genenames.org/ <u>HUGO-approved gene names and symbols</u>] (italicized when appropriate), [https://varnomen.hgvs.org/ HGVS-based nomenclature for variants], as well as generic names of drugs and testing platforms or assays if applicable. Please complete tables whenever possible and do not delete them (add N/A if not applicable in the table and delete the examples); to add (or move) a row or column to a table, click nearby within the table and select the > symbol that appears to be given options. Please do not delete or alter the section headings. The use of bullet points alongside short blocks of text rather than only large paragraphs is encouraged. Additional instructions below in italicized blue text should not be included in the final page content. Please also see'' </span><u>''[[Author_Instructions]]''</u><span style="color:#0070C0"> ''and [[Frequently Asked Questions (FAQs)|<u>FAQs</u>]] as well as contact your [[Leadership|<u>Associate Editor</u>]] or [mailto:CCGA@cancergenomics.org <u>Technical Support</u>])''</span>

<span style="color:#0070C0">(''General Instructions – The main focus of these pages is the clinically significant genetic alterations in each disease type. Use [https://www.genenames.org/ <u>HUGO-approved gene names and symbols</u>] (italicized when appropriate), [https://varnomen.hgvs.org/ HGVS-based nomenclature for variants], as well as generic names of drugs and testing platforms or assays if applicable. Please complete tables whenever possible and do not delete them (add N/A if not applicable in the table and delete the examples); to add (or move) a row or column to a table, click nearby within the table and select the > symbol that appears to be given options. Please do not delete or alter the section headings. The use of bullet points alongside short blocks of text rather than only large paragraphs is encouraged. Additional instructions below in italicized blue text should not be included in the final page content. Please also see'' </span><u>''[[Author_Instructions]]''</u><span style="color:#0070C0"> ''and [[Frequently Asked Questions (FAQs)|<u>FAQs</u>]] as well as contact your [[Leadership|<u>Associate Editor</u>]] or [mailto:CCGA@cancergenomics.org <u>Technical Support</u>])''</span>

==Primary Author(s)*==

==Primary Author(s)*==

<span style="color:#0070C0">Katherine Geiersbach, M.D., Ganesh P. Pujari, M.D, FICC </span>

<span style="color:#0070C0">Ganesh P. Pujari, M.D, FICC and Katherine Geiersbach, M.D.</span>

==WHO Classification of Disease==

==WHO Classification of Disease==

<span style="color:#0070C0">(Will be autogenerated; Book will include name of specific book and have a link to the online WHO site)</span>

<span style="color:#0070C0">(Will be autogenerated; Book will include name of specific book and have a link to the online WHO site)</span>

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==Definition / Description of Disease==

==Definition / Description of Disease==

Put your text here <span style="color:#0070C0">(''Instructions: Brief description of approximately one paragraph - include disease context relative to other WHO classification categories, diagnostic criteria if applicable, and differential diagnosis if applicable. Other classifications can be referenced for comparison.'') </span>

Previously grouped into Ewing and other primitive sarcoma categories, it was not until 2006 that the CIC-rearranged sarcomas were identified as a distinct subgroup of soft tissue neoplasms in the “Ewing-like sarcomas” category. Apart from the classical DUX4, approximately 8 other partner genes have been reported so far. <ref>{{Cite journal|last=Makise|first=Naohiro|last2=Yoshida|first2=Akihiko|date=2024-03|title=CIC-Rearranged Sarcoma|url=https://pubmed.ncbi.nlm.nih.gov/38278603|journal=Surgical Pathology Clinics|volume=17|issue=1|pages=141–151|doi=10.1016/j.path.2023.06.003|issn=1875-9157|pmid=38278603}}</ref> Overall due to its characteristic histomorphologic and immunophenotypic features, diagnostic molecular rearrangements, highly aggressive clinical course, poor response to chemotherapy and adverse outcome (compared to Ewing sarcoma) CIC-rearranged sarcoma has been recognized as a distinct pathologic entity by the WHO under the category of undifferentiated small round cell sarcomas of bone and soft tissue and grouped along with the sarcoma with BCOR genetic alterations, and round cell sarcoma with EWSR1::non-ETS fusions. <ref>WHO Classification of Tumours Editorial Board. Soft tissue and bone tumours. Lyon (France): International Agency for Research on Cancer; 2020. (WHO classification of tumours series, 5th ed.; vol. 3). <nowiki>https://publications.iarc.fr/588</nowiki>.</ref>

==Synonyms / Terminology==

==Synonyms / Terminology==

Put your text here <span style="color:#0070C0">(''Instructions: Include currently used terms and major historical ones, adding “(historical)” after the latter.'') </span>

Put your text here <span style="color:#0070C0">(''Instructions: Include currently used terms and major historical ones, adding “(historical)” after the latter.'') </span>