A Rare Case of Nasal Sarcoma with BCOR Internal Tandem Duplication Showing Complete Pathologic Response to the VDC-IE Chemotherapy Protocol
Sarcoma with BCOR genetic alteration is an exceptionally rare and emerging subtype of sarcoma. It is categorized into two types: BCOR-related gene fusions such as BCOR::CCNB3 sarcomas and other BCOR-rearranged sarcoma and sarcomas with internal tandem duplication of BCOR genes such as infantile undi...
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| Format: | Article |
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Wiley
2023-01-01
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| Series: | Case Reports in Oncological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2023/5546323 |
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| author | Samer Salah Maher A. Sughayer Omar Jaber Nebras Abu Abed Fatena Ajlouni Wisam Al Gargaz Ramiz Abu Hijlih Fawzi Abuhijla Akram Al-Ibraheem Farah Alul Walid Naser |
| author_facet | Samer Salah Maher A. Sughayer Omar Jaber Nebras Abu Abed Fatena Ajlouni Wisam Al Gargaz Ramiz Abu Hijlih Fawzi Abuhijla Akram Al-Ibraheem Farah Alul Walid Naser |
| author_sort | Samer Salah |
| collection | DOAJ |
| description | Sarcoma with BCOR genetic alteration is an exceptionally rare and emerging subtype of sarcoma. It is categorized into two types: BCOR-related gene fusions such as BCOR::CCNB3 sarcomas and other BCOR-rearranged sarcoma and sarcomas with internal tandem duplication of BCOR genes such as infantile undifferentiated round cell sarcomas and primitive myxoid mesenchymal tumors of infancy. BCOR::CCNB3 sarcomas predominantly arise in bone rather than soft tissue and exhibit a higher occurrence in children and adolescent males, whereas sarcomas with BCOR internal tandem duplication show a wider age range but usually arise in the first year of life. Due to their rarity, there is ongoing debate and uncertainty regarding the best treatment approach, with a lack of specific clinical trials addressing these tumors. In this report, we present a unique case of sarcoma with internal tandem duplication of BCOR gene originating in the nasal region. The tumor was successfully and completely resected using the standard VDC-IE chemotherapy protocol, resulting in an unprecedented 100 percent tumor necrosis. The patient has completed the protocol and remains recurrence-free 13 months after diagnosis. This case suggests potential efficacy of the standard VDC-IE protocol in achieving remarkable responses in BCOR rearrangement sarcomas, including the internal tandem duplication subtype. However, further studies are needed to determine the optimal treatment strategies for this disease. |
| format | Article |
| id | doaj-art-773a610f231b4444bd3ca68a028f039b |
| institution | OA Journals |
| issn | 2090-6714 |
| language | English |
| publishDate | 2023-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Oncological Medicine |
| spelling | doaj-art-773a610f231b4444bd3ca68a028f039b2025-08-20T02:07:34ZengWileyCase Reports in Oncological Medicine2090-67142023-01-01202310.1155/2023/5546323A Rare Case of Nasal Sarcoma with BCOR Internal Tandem Duplication Showing Complete Pathologic Response to the VDC-IE Chemotherapy ProtocolSamer Salah0Maher A. Sughayer1Omar Jaber2Nebras Abu Abed3Fatena Ajlouni4Wisam Al Gargaz5Ramiz Abu Hijlih6Fawzi Abuhijla7Akram Al-Ibraheem8Farah Alul9Walid Naser10Department of Medical OncologyDepartment of PathologyDepartment of PathologyDepartment of Medical OncologyDepartment of RadiologyDepartment of SurgeryDepartment of Radiation OncologyDepartment of Radiation OncologyDepartment of Nuclear MedicineDepartment of Cell Therapy and Applied GenomicsDepartment of Cell Therapy and Applied GenomicsSarcoma with BCOR genetic alteration is an exceptionally rare and emerging subtype of sarcoma. It is categorized into two types: BCOR-related gene fusions such as BCOR::CCNB3 sarcomas and other BCOR-rearranged sarcoma and sarcomas with internal tandem duplication of BCOR genes such as infantile undifferentiated round cell sarcomas and primitive myxoid mesenchymal tumors of infancy. BCOR::CCNB3 sarcomas predominantly arise in bone rather than soft tissue and exhibit a higher occurrence in children and adolescent males, whereas sarcomas with BCOR internal tandem duplication show a wider age range but usually arise in the first year of life. Due to their rarity, there is ongoing debate and uncertainty regarding the best treatment approach, with a lack of specific clinical trials addressing these tumors. In this report, we present a unique case of sarcoma with internal tandem duplication of BCOR gene originating in the nasal region. The tumor was successfully and completely resected using the standard VDC-IE chemotherapy protocol, resulting in an unprecedented 100 percent tumor necrosis. The patient has completed the protocol and remains recurrence-free 13 months after diagnosis. This case suggests potential efficacy of the standard VDC-IE protocol in achieving remarkable responses in BCOR rearrangement sarcomas, including the internal tandem duplication subtype. However, further studies are needed to determine the optimal treatment strategies for this disease.http://dx.doi.org/10.1155/2023/5546323 |
| spellingShingle | Samer Salah Maher A. Sughayer Omar Jaber Nebras Abu Abed Fatena Ajlouni Wisam Al Gargaz Ramiz Abu Hijlih Fawzi Abuhijla Akram Al-Ibraheem Farah Alul Walid Naser A Rare Case of Nasal Sarcoma with BCOR Internal Tandem Duplication Showing Complete Pathologic Response to the VDC-IE Chemotherapy Protocol Case Reports in Oncological Medicine |
| title | A Rare Case of Nasal Sarcoma with BCOR Internal Tandem Duplication Showing Complete Pathologic Response to the VDC-IE Chemotherapy Protocol |
| title_full | A Rare Case of Nasal Sarcoma with BCOR Internal Tandem Duplication Showing Complete Pathologic Response to the VDC-IE Chemotherapy Protocol |
| title_fullStr | A Rare Case of Nasal Sarcoma with BCOR Internal Tandem Duplication Showing Complete Pathologic Response to the VDC-IE Chemotherapy Protocol |
| title_full_unstemmed | A Rare Case of Nasal Sarcoma with BCOR Internal Tandem Duplication Showing Complete Pathologic Response to the VDC-IE Chemotherapy Protocol |
| title_short | A Rare Case of Nasal Sarcoma with BCOR Internal Tandem Duplication Showing Complete Pathologic Response to the VDC-IE Chemotherapy Protocol |
| title_sort | rare case of nasal sarcoma with bcor internal tandem duplication showing complete pathologic response to the vdc ie chemotherapy protocol |
| url | http://dx.doi.org/10.1155/2023/5546323 |
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