Expanding the Spectrum of EWSR1::CREM Fusion Tumors: An Unusual Pediatric Intranasal Myxoid Tumor
EWSR1::CREM gene fusions are increasingly being recognized in a diverse number of soft tissue tumors, including well-defined entities such as angiomatoid fibrous histiocytoma or clear cell sarcoma, and other unclassifiable tumors. As a group, EWSR1::CREM fused tumors often demonstrate primitive spin...
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| Veröffentlicht in: | Pediatric and developmental pathology 2024-01, Vol.27 (1), p.90-95 |
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| container_title | Pediatric and developmental pathology |
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| creator | Koh, Shamen Punjabi, Lavisha S. Chang, Kenneth Tou En Wei Yang Teo, Neville Ee Hoon Teo, Constance Soh, Shui Yen Kun Kiaang Tan, Henry |
| description | EWSR1::CREM gene fusions are increasingly being recognized in a diverse number of soft tissue tumors, including well-defined entities such as angiomatoid fibrous histiocytoma or clear cell sarcoma, and other unclassifiable tumors. As a group, EWSR1::CREM fused tumors often demonstrate primitive spindle or epithelioid cells, myxoid stroma, and a broad immunophenotype. Herein we present an unusual case of a child diagnosed with an intranasal malignant myxoid tumor harboring an EWSR1::CREM gene fusion. To the best of our knowledge, this is the first case of intranasal myxoid tumor with this particular fusion. Diagnosis and management of the case is discussed. |
| doi_str_mv | 10.1177/10935266231199931 |
| format | Article |
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Diagnosis and management of the case is discussed.</description><identifier>ISSN: 1093-5266</identifier><identifier>EISSN: 1615-5742</identifier><identifier>DOI: 10.1177/10935266231199931</identifier><identifier>PMID: 37818644</identifier><language>eng</language><publisher>Los Angeles, CA: SAGE Publications</publisher><subject>Biomarkers, Tumor - genetics ; Child ; Cyclic AMP Response Element Modulator - genetics ; Gene Fusion ; Histiocytoma, Malignant Fibrous - genetics ; Humans ; Oncogene Proteins, Fusion - genetics ; RNA-Binding Protein EWS - genetics ; Sarcoma, Clear Cell ; Soft Tissue Neoplasms - diagnosis ; Soft Tissue Neoplasms - genetics ; Soft Tissue Neoplasms - pathology</subject><ispartof>Pediatric and developmental pathology, 2024-01, Vol.27 (1), p.90-95</ispartof><rights>2023, Society for Pediatric Pathology All rights reserved</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c292t-d7bfa751310eb94aab73a3856516c94b527fc22c70b29618bbff55444c5c2d1a3</cites><orcidid>0000-0001-5244-4285 ; 0009-0000-0882-3130</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://journals.sagepub.com/doi/pdf/10.1177/10935266231199931$$EPDF$$P50$$Gsage$$H</linktopdf><linktohtml>$$Uhttps://journals.sagepub.com/doi/10.1177/10935266231199931$$EHTML$$P50$$Gsage$$H</linktohtml><link.rule.ids>314,780,784,21819,27924,27925,43621,43622</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/37818644$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Koh, Shamen</creatorcontrib><creatorcontrib>Punjabi, Lavisha S.</creatorcontrib><creatorcontrib>Chang, Kenneth Tou En</creatorcontrib><creatorcontrib>Wei Yang Teo, Neville</creatorcontrib><creatorcontrib>Ee Hoon Teo, Constance</creatorcontrib><creatorcontrib>Soh, Shui Yen</creatorcontrib><creatorcontrib>Kun Kiaang Tan, Henry</creatorcontrib><title>Expanding the Spectrum of EWSR1::CREM Fusion Tumors: An Unusual Pediatric Intranasal Myxoid Tumor</title><title>Pediatric and developmental pathology</title><addtitle>Pediatr Dev Pathol</addtitle><description>EWSR1::CREM gene fusions are increasingly being recognized in a diverse number of soft tissue tumors, including well-defined entities such as angiomatoid fibrous histiocytoma or clear cell sarcoma, and other unclassifiable tumors. 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Diagnosis and management of the case is discussed.</description><subject>Biomarkers, Tumor - genetics</subject><subject>Child</subject><subject>Cyclic AMP Response Element Modulator - genetics</subject><subject>Gene Fusion</subject><subject>Histiocytoma, Malignant Fibrous - genetics</subject><subject>Humans</subject><subject>Oncogene Proteins, Fusion - genetics</subject><subject>RNA-Binding Protein EWS - genetics</subject><subject>Sarcoma, Clear Cell</subject><subject>Soft Tissue Neoplasms - diagnosis</subject><subject>Soft Tissue Neoplasms - genetics</subject><subject>Soft Tissue Neoplasms - pathology</subject><issn>1093-5266</issn><issn>1615-5742</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kF9LwzAUxYMobk4_gC-SR186e9Okafc2RqcDh7I_-FjSNJ0dazqTBrZvb0enL4JP93Lv7xw4B6F78IcAnD-BHweMhCEJAOI4DuAC9SEE5jFOyWW7t3_vBPTQjbVb329FoX-NegGPIAop7SORHPZC56Xe4OZT4eVeyca4CtcFTj6WCxiNJotkjqfOlrXGK1fVxo7wWOO1dtaJHX5XeSkaU0o8040RWtj2OD8e6jLv8Ft0VYidVXfnOUDrabKavHivb8-zyfjVkyQmjZfzrBCcQQC-ymIqRMYDEUQsZBDKmGaM8EISIrmfkTiEKMuKgjFKqWSS5CCCAXrsfPem_nLKNmlVWql2O6FV7WxKIs4iBpRHLQodKk1trVFFujdlJcwxBT89NZv-abbVPJztXVap_FfxU2ULDDvAio1Kt7Uzuo37j-M3K2d_YQ</recordid><startdate>202401</startdate><enddate>202401</enddate><creator>Koh, Shamen</creator><creator>Punjabi, Lavisha S.</creator><creator>Chang, Kenneth Tou En</creator><creator>Wei Yang Teo, Neville</creator><creator>Ee Hoon Teo, Constance</creator><creator>Soh, Shui Yen</creator><creator>Kun Kiaang Tan, Henry</creator><general>SAGE Publications</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0001-5244-4285</orcidid><orcidid>https://orcid.org/0009-0000-0882-3130</orcidid></search><sort><creationdate>202401</creationdate><title>Expanding the Spectrum of EWSR1::CREM Fusion Tumors: An Unusual Pediatric Intranasal Myxoid Tumor</title><author>Koh, Shamen ; Punjabi, Lavisha S. ; Chang, Kenneth Tou En ; Wei Yang Teo, Neville ; Ee Hoon Teo, Constance ; Soh, Shui Yen ; Kun Kiaang Tan, Henry</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c292t-d7bfa751310eb94aab73a3856516c94b527fc22c70b29618bbff55444c5c2d1a3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>Biomarkers, Tumor - genetics</topic><topic>Child</topic><topic>Cyclic AMP Response Element Modulator - genetics</topic><topic>Gene Fusion</topic><topic>Histiocytoma, Malignant Fibrous - genetics</topic><topic>Humans</topic><topic>Oncogene Proteins, Fusion - genetics</topic><topic>RNA-Binding Protein EWS - genetics</topic><topic>Sarcoma, Clear Cell</topic><topic>Soft Tissue Neoplasms - diagnosis</topic><topic>Soft Tissue Neoplasms - genetics</topic><topic>Soft Tissue Neoplasms - pathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Koh, Shamen</creatorcontrib><creatorcontrib>Punjabi, Lavisha S.</creatorcontrib><creatorcontrib>Chang, Kenneth Tou En</creatorcontrib><creatorcontrib>Wei Yang Teo, Neville</creatorcontrib><creatorcontrib>Ee Hoon Teo, Constance</creatorcontrib><creatorcontrib>Soh, Shui Yen</creatorcontrib><creatorcontrib>Kun Kiaang Tan, Henry</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric and developmental pathology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Koh, Shamen</au><au>Punjabi, Lavisha S.</au><au>Chang, Kenneth Tou En</au><au>Wei Yang Teo, Neville</au><au>Ee Hoon Teo, Constance</au><au>Soh, Shui Yen</au><au>Kun Kiaang Tan, Henry</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Expanding the Spectrum of EWSR1::CREM Fusion Tumors: An Unusual Pediatric Intranasal Myxoid Tumor</atitle><jtitle>Pediatric and developmental pathology</jtitle><addtitle>Pediatr Dev Pathol</addtitle><date>2024-01</date><risdate>2024</risdate><volume>27</volume><issue>1</issue><spage>90</spage><epage>95</epage><pages>90-95</pages><issn>1093-5266</issn><eissn>1615-5742</eissn><abstract>EWSR1::CREM gene fusions are increasingly being recognized in a diverse number of soft tissue tumors, including well-defined entities such as angiomatoid fibrous histiocytoma or clear cell sarcoma, and other unclassifiable tumors. 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| subjects | Biomarkers, Tumor - genetics Child Cyclic AMP Response Element Modulator - genetics Gene Fusion Histiocytoma, Malignant Fibrous - genetics Humans Oncogene Proteins, Fusion - genetics RNA-Binding Protein EWS - genetics Sarcoma, Clear Cell Soft Tissue Neoplasms - diagnosis Soft Tissue Neoplasms - genetics Soft Tissue Neoplasms - pathology |
| title | Expanding the Spectrum of EWSR1::CREM Fusion Tumors: An Unusual Pediatric Intranasal Myxoid Tumor |
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