STBT5:CIC-rearranged sarcoma: Difference between revisions - Compendium of Cancer Genome Aberrations

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==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==

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

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