STBT5:CIC-rearranged sarcoma - Revision history

Richard.Glen at 00:52, 26 February 2026

2026-02-26T00:52:26Z

← Older revision		Revision as of 19:52, 25 February 2026
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	==References==		==References==
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			<references />

	==Notes==		==Notes==

Richard.Glen at 00:48, 26 February 2026

2026-02-26T00:48:14Z

← Older revision		Revision as of 19:48, 25 February 2026
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	Put a link here or anywhere appropriate in this page <span style="color:#0070C0">(''Instructions: Highlight the text to which you want to add a link in this section or elsewhere, select the "Link" icon at the top of the wiki page, and search the name of the internal page to which you want to link this text, or enter an external internet address by including the "<nowiki>http://www</nowiki>." portion.'')</span>		Put a link here or anywhere appropriate in this page <span style="color:#0070C0">(''Instructions: Highlight the text to which you want to add a link in this section or elsewhere, select the "Link" icon at the top of the wiki page, and search the name of the internal page to which you want to link this text, or enter an external internet address by including the "<nowiki>http://www</nowiki>." portion.'')</span>
	==References==		==References==
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	==Notes==		==Notes==
	<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the [[Leadership\|''<u>Associate Editor</u>'']] or other CCGA representative. When pages have a major update, the new author will be acknowledged at the beginning of the page, and those who contributed previously will be acknowledged below as a prior author.		<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the [[Leadership\|''<u>Associate Editor</u>'']] or other CCGA representative. When pages have a major update, the new author will be acknowledged at the beginning of the page, and those who contributed previously will be acknowledged below as a prior author.

Richard.Glen at 18:37, 3 February 2026

2026-02-03T18:37:48Z

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Richard.Glen: Removed redirect to CNS5:CIC-rearranged sarcoma

2026-02-03T18:35:59Z

Removed redirect to CNS5:CIC-rearranged sarcoma

← Older revision		Revision as of 13:35, 3 February 2026
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	~~#REDIRECT[[CNS5~~:~~CIC-rearranged sarcoma~~]]		ory:Diseases C]]"}}}

Richard.Glen: Redirected page to CNS5:CIC-rearranged sarcoma

2026-02-03T18:30:14Z

Redirected page to CNS5:CIC-rearranged sarcoma

Show changes

Bailey.Glen at 17:56, 3 July 2025

2025-07-03T17:56:57Z

← Older revision		Revision as of 12:56, 3 July 2025
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	\|}		\|}

	~~==WHO Essential and Desirable Genetic Diagnostic Criteria==~~
	<span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span>
	~~{\| class="wikitable"~~
	\|+
	\|WHO Essential Criteria (Genetics)*
	\|
	\|-
	\|WHO Desirable Criteria (Genetics)*
	\|
	\|-
	~~\|Other Classification~~
	\|
	\|}
	<nowiki>*</nowiki>Note: These are only the genetic/genomic criteria. Additional diagnostic criteria can be found in the [https://tumourclassification.iarc.who.int/home <u>WHO Classification of Tumours</u>].
	==Related Terminology==		==Related Terminology==

Bailey.Glen at 22:17, 25 May 2025

2025-05-25T22:17:19Z

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	{\| class="wikitable sortable"		{\| class="wikitable sortable"
	\|-		\|-
	!Chr #!!~~'''~~Gain, Loss, Amp, LOH~~'''~~!!~~'''~~Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]~~'''~~!!~~'''~~Relevant Gene(s)~~'''~~		!Chr #!!Gain, Loss, Amp, LOH!!Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]!!Relevant Gene(s)
	!~~'''~~Diagnostic, Prognostic, and Therapeutic Significance - D, P, T~~'''~~		!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
	!~~'''~~Established Clinical Significance Per Guidelines - Yes or No (Source)~~'''~~		!Established Clinical Significance Per Guidelines - Yes or No (Source)
	!~~'''~~Clinical Relevance Details/Other Notes~~'''~~		!Clinical Relevance Details/Other Notes
	\|-		\|-
	\|<span class="blue-text">EXAMPLE:</span>		\|<span class="blue-text">EXAMPLE:</span>
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	!Chromosomal Pattern		!Chromosomal Pattern
	!Molecular Pathogenesis		!Molecular Pathogenesis
	!~~'''~~Prevalence -~~'''~~		!Prevalence -
	~~'''~~Common >20%, Recurrent 5-20% or Rare <5% (Disease)~~'''~~		Common >20%, Recurrent 5-20% or Rare <5% (Disease)
	!~~'''~~Diagnostic, Prognostic, and Therapeutic Significance - D, P, T~~'''~~		!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
	!~~'''~~Established Clinical Significance Per Guidelines - Yes or No (Source)~~'''~~		!Established Clinical Significance Per Guidelines - Yes or No (Source)
	!~~'''~~Clinical Relevance Details/Other Notes~~'''~~		!Clinical Relevance Details/Other Notes
	\|-		\|-
	\|<span class="blue-text">EXAMPLE:</span>		\|<span class="blue-text">EXAMPLE:</span>
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	{\| class="wikitable sortable"		{\| class="wikitable sortable"
	\|-		\|-
	!Gene!!~~'''~~Genetic Alteration~~'''~~!!~~'''~~Tumor Suppressor Gene, Oncogene, Other~~'''~~!!~~'''~~Prevalence -~~'''~~		!Gene!!Genetic Alteration!!Tumor Suppressor Gene, Oncogene, Other!!Prevalence -
	~~'''~~Common >20%, Recurrent 5-20% or Rare <5% (Disease)~~'''~~		Common >20%, Recurrent 5-20% or Rare <5% (Disease)
	!~~'''~~Diagnostic, Prognostic, and Therapeutic Significance - D, P, T ~~'''~~		!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
	!~~'''~~Established Clinical Significance Per Guidelines - Yes or No (Source)~~'''~~		!Established Clinical Significance Per Guidelines - Yes or No (Source)
	!~~'''~~Clinical Relevance Details/Other Notes~~'''~~		!Clinical Relevance Details/Other Notes
	\|-		\|-
	\|<span class="blue-text">EXAMPLE:</span>''EGFR''		\|<span class="blue-text">EXAMPLE:</span>''EGFR''

Bailey.Glen at 20:53, 25 May 2025

2025-05-25T20:53:00Z

← Older revision		Revision as of 15:53, 25 May 2025
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	<nowiki>*</nowiki>Note: These are only the genetic/genomic criteria. Additional diagnostic criteria can be found in the [https://tumourclassification.iarc.who.int/home <u>WHO Classification of Tumours</u>].		<nowiki>*</nowiki>Note: These are only the genetic/genomic criteria. Additional diagnostic criteria can be found in the [https://tumourclassification.iarc.who.int/home <u>WHO Classification of Tumours</u>].
	==Related Terminology==		==Related Terminology==
	~~<span style="color:#0070C0">(''Instructions: The table will have the related terminology from the WHO <u>autocompleted</u>.)''</span>~~
	{\| class="wikitable"		{\| class="wikitable"
	\|+		\|+
	\|Acceptable		\|Acceptable
	\|		\|CIC-DUX4 sarcoma
	\|-		\|-
	\|Not Recommended		\|Not Recommended
	\|		\|N/A
	\|}		\|}

Bailey.Glen at 19:09, 25 May 2025

2025-05-25T19:09:49Z

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Kgeiersbach at 19:19, 14 March 2024

2024-03-14T19:19:00Z

← Older revision		Revision as of 14:19, 14 March 2024
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	\|}		\|}
	==Definition / Description of Disease==		==Definition / Description of Disease==
	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 {Makise, 2024 #1}. 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 {Board, ~~2020 #2}~~.		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>