Primary renal sarcoma in a female patient: case report
BCOR-rearranged sarcoma was classified by the World Health Organization in 2020 as a new subgroup of undifferentiated small round-cell sarcoma. It is known to occur very rarely in the kidney. This report presents the first case of a primary renal BCOR::CCNB3 sarcoma in a 22-year-old woman. An 8-cm c...
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Korean Society of Pathologists & the Korean Society for Cytopathology
2025-01-01
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| Series: | Journal of Pathology and Translational Medicine |
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| Online Access: | http://www.jpatholtm.org/upload/pdf/jptm-2024-09-30.pdf |
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| author | Somang Lee Binnari Kim |
| author_facet | Somang Lee Binnari Kim |
| author_sort | Somang Lee |
| collection | DOAJ |
| description | BCOR-rearranged sarcoma was classified by the World Health Organization in 2020 as a new subgroup of undifferentiated small round-cell sarcoma. It is known to occur very rarely in the kidney. This report presents the first case of a primary renal BCOR::CCNB3 sarcoma in a 22-year-old woman. An 8-cm cystic mass was identified in the left kidney by abdominal pelvic computed tomography. Histopathologic examination revealed the mass to be composed of small round to oval or spindle cells with fibrous septa and a delicate vascular network. A BCOR::CCNB3 fusion was detected by next-generation sequencing–based molecular testing. BCOR::CCNB3 sarcoma presents diagnostic difficulties, highlighting the importance of recognizing its histological features. Immunohistochemical markers are helpful for diagnosis, but genetic molecular testing is necessary for accurate diagnosis. These tumors have a very poor and aggressive prognosis, and an optimal therapeutic regimen has not yet been defined. Therefore, further studies are needed. |
| format | Article |
| id | doaj-art-84998193d98a41839d3e46112b8a1064 |
| institution | Kabale University |
| issn | 2383-7837 2383-7845 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Korean Society of Pathologists & the Korean Society for Cytopathology |
| record_format | Article |
| series | Journal of Pathology and Translational Medicine |
| spelling | doaj-art-84998193d98a41839d3e46112b8a10642025-01-16T08:11:40ZengKorean Society of Pathologists & the Korean Society for CytopathologyJournal of Pathology and Translational Medicine2383-78372383-78452025-01-01591849010.4132/jptm.2024.09.3017138Primary renal sarcoma in a female patient: case reportSomang Lee0Binnari Kim1 Department of Pathology, Ulsan University Hospital, Ulsan, Korea Department of Pathology, Ulsan University Hospital, Ulsan, KoreaBCOR-rearranged sarcoma was classified by the World Health Organization in 2020 as a new subgroup of undifferentiated small round-cell sarcoma. It is known to occur very rarely in the kidney. This report presents the first case of a primary renal BCOR::CCNB3 sarcoma in a 22-year-old woman. An 8-cm cystic mass was identified in the left kidney by abdominal pelvic computed tomography. Histopathologic examination revealed the mass to be composed of small round to oval or spindle cells with fibrous septa and a delicate vascular network. A BCOR::CCNB3 fusion was detected by next-generation sequencing–based molecular testing. BCOR::CCNB3 sarcoma presents diagnostic difficulties, highlighting the importance of recognizing its histological features. Immunohistochemical markers are helpful for diagnosis, but genetic molecular testing is necessary for accurate diagnosis. These tumors have a very poor and aggressive prognosis, and an optimal therapeutic regimen has not yet been defined. Therefore, further studies are needed.http://www.jpatholtm.org/upload/pdf/jptm-2024-09-30.pdfadultbiomarkers, tumorfemalegene fusionimmunogistochemistrykidneysarcoma |
| spellingShingle | Somang Lee Binnari Kim Primary renal sarcoma in a female patient: case report Journal of Pathology and Translational Medicine adult biomarkers, tumor female gene fusion immunogistochemistry kidney sarcoma |
| title | Primary renal sarcoma in a female patient: case report |
| title_full | Primary renal sarcoma in a female patient: case report |
| title_fullStr | Primary renal sarcoma in a female patient: case report |
| title_full_unstemmed | Primary renal sarcoma in a female patient: case report |
| title_short | Primary renal sarcoma in a female patient: case report |
| title_sort | primary renal sarcoma in a female patient case report |
| topic | adult biomarkers, tumor female gene fusion immunogistochemistry kidney sarcoma |
| url | http://www.jpatholtm.org/upload/pdf/jptm-2024-09-30.pdf |
| work_keys_str_mv | AT somanglee primaryrenalsarcomainafemalepatientcasereport AT binnarikim primaryrenalsarcomainafemalepatientcasereport |