{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T21:58:28Z","timestamp":1791928708005,"version":"4.3.4"},"reference-count":56,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2017,10,30]],"date-time":"2017-10-30T00:00:00Z","timestamp":1509321600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"VIVA-KKH Paediatric Brain and Solid Tumours Programme"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"published-print":{"date-parts":[[2018,1]]},"abstract":"Aims<\/jats:title>Clear cell sarcoma of the kidney (CCSK<\/jats:styled-content>) is a rare paediatric renal malignant tumour. The majority of CCSK<\/jats:styled-content>s have internal tandem duplications (ITD<\/jats:styled-content>s) of the BCOR<\/jats:styled-content><\/jats:italic> gene, whereas a minority have the YWHAE<\/jats:styled-content>\u2013NUTM<\/jats:styled-content>2<\/jats:italic> gene fusion. A third \u2018double\u2010negative\u2019 (DN<\/jats:styled-content>) category comprises CCSK<\/jats:styled-content>s with neither BCOR<\/jats:styled-content><\/jats:italic> ITD<\/jats:styled-content>s nor YWHAE<\/jats:styled-content>\u2013NUTM<\/jats:styled-content>2<\/jats:italic> fusion. The aim of this study was to characterise 11 histologically diagnosed CCSK<\/jats:styled-content>s immunohistochemically (with CCND<\/jats:styled-content>1, BCOR<\/jats:styled-content> and CCNB<\/jats:styled-content>3 stains) and genetically.<\/jats:p><\/jats:sec>Methods and results<\/jats:title>By next\u2010generation sequencing, 10 cases (90.9%) had BCOR<\/jats:styled-content><\/jats:italic> exon 15 ITD<\/jats:styled-content>s, with positive BCOR<\/jats:styled-content> immunoreactivity being found in four (36%) or eight (72%) cases, depending on the antibody clone. By reverse transcription polymerase chain reaction, none had the YWHAE<\/jats:styled-content>\u2013NUTM<\/jats:styled-content>2<\/jats:italic> fusion. The DN<\/jats:styled-content> case had a BCOR<\/jats:styled-content>\u2013CCNB<\/jats:styled-content>3<\/jats:italic> fusion and strong nuclear CCNB<\/jats:styled-content>3 and BCOR<\/jats:styled-content> immunoreactivity. Quantitative polymerase chain reaction showed markedly elevated BCOR<\/jats:styled-content><\/jats:italic> expression in this case, whereas BCOR<\/jats:styled-content><\/jats:italic> ITD<\/jats:styled-content> cases had lower levels of elevated BCOR<\/jats:styled-content><\/jats:italic> expression.<\/jats:p><\/jats:sec>Conclusions<\/jats:title>The majority of the CCSK<\/jats:styled-content>s in our cohort had BCOR<\/jats:styled-content><\/jats:italic> ITD<\/jats:styled-content>s, and none had the YWHAE<\/jats:styled-content>\u2013NUTM<\/jats:styled-content>2<\/jats:italic> fusion. We verified the strong, diffuse cyclin D1 (CCND<\/jats:styled-content>1) immunoreactivity in CCSK<\/jats:styled-content>s described in recent reports. BCOR<\/jats:styled-content> immunoreactivity was not consistently positive in all CCSK<\/jats:styled-content>s with BCOR<\/jats:styled-content><\/jats:italic> ITD<\/jats:styled-content>s, and therefore cannot be used as a diagnostic immunohistochemical stain to identify BCOR<\/jats:styled-content><\/jats:italic> ITD<\/jats:styled-content> cases. The DN<\/jats:styled-content> case was a BCOR<\/jats:styled-content>\u2013CCNB<\/jats:styled-content>3<\/jats:italic> fusion sarcoma. BCOR<\/jats:styled-content>\u2013CCNB<\/jats:styled-content>3<\/jats:italic> sarcoma is typically a primary bone sarcoma affecting male adolescents, and this is the first report of it presenting in a kidney of a young child as a CCSK<\/jats:styled-content>. The full spectrum of DN CCSK<\/jats:styled-content>s awaits more comprehensive characterisation.<\/jats:p><\/jats:sec>","DOI":"10.1111\/his.13366","type":"journal-article","created":{"date-parts":[[2017,8,18]],"date-time":"2017-08-18T15:02:34Z","timestamp":1503068554000},"page":"320-329","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":74,"title":["Clear cell sarcomas of the kidney are characterised by BCOR<\/scp><\/i> gene abnormalities, including exon 15 internal tandem duplications and BCOR<\/scp>\u2013CCNB<\/scp>3<\/i> gene fusion"],"prefix":"10.1111","volume":"72","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0496-7844","authenticated-orcid":false,"given":"Meng K","family":"Wong","sequence":"first","affiliation":[{"name":"VIVA\u2010KKH Paediatric Brain and Solid Tumour Laboratory KK Women's and Children's Hospital Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cedric C Y","family":"Ng","sequence":"additional","affiliation":[{"name":"Laboratory of Cancer Epigenome National Cancer Centre Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chik H","family":"Kuick","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine KK Women's and Children's Hospital Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sze J","family":"Aw","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine KK Women's and Children's Hospital Singapore"},{"name":"Department of Anatomical Pathology Singapore General Hospital Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vikneswari","family":"Rajasegaran","sequence":"additional","affiliation":[{"name":"Laboratory of Cancer Epigenome National Cancer Centre Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jing Q","family":"Lim","sequence":"additional","affiliation":[{"name":"Laboratory of Cancer Epigenome National Cancer Centre Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jain","family":"Sudhanshi","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine KK Women's and Children's Hospital Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eva","family":"Loh","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine KK Women's and Children's Hospital Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minzhi","family":"Yin","sequence":"additional","affiliation":[{"name":"Department of Pathology Shanghai Children's Medical Centre Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jing","family":"Ma","sequence":"additional","affiliation":[{"name":"Department of Pathology Shanghai Children's Medical Centre Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhongde","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pathology Shanghai Children's Medical Centre Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Prasad","family":"Iyer","sequence":"additional","affiliation":[{"name":"Paediatric Haematology\u2010Oncology Service Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amos H P","family":"Loh","sequence":"additional","affiliation":[{"name":"VIVA\u2010KKH Paediatric Brain and Solid Tumour Laboratory KK Women's and Children's Hospital Singapore"},{"name":"Department of Paediatric Surgery KK Women's and Children's Hospital Singapore"},{"name":"Duke\u2010NUS Medical School Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Derrick W Q","family":"Lian","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine KK Women's and Children's Hospital Singapore"},{"name":"Duke\u2010NUS Medical School Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shi","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pathology National University Health System Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shaun G H","family":"Goh","sequence":"additional","affiliation":[{"name":"Department of Pathology National University Health System Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tse H","family":"Lim","sequence":"additional","affiliation":[{"name":"Department of Molecular Pathology Singapore General Hospital Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alvin S T","family":"Lim","sequence":"additional","affiliation":[{"name":"Department of Molecular Pathology Singapore General Hospital Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tony","family":"Ng","sequence":"additional","affiliation":[{"name":"Department of Pathology Vancouver General Hospital and University of British Columbia Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Goytain","sequence":"additional","affiliation":[{"name":"Department of Pathology Vancouver General Hospital and University of British Columbia Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alwin H L","family":"Loh","sequence":"additional","affiliation":[{"name":"Department of Anatomical Pathology Singapore General Hospital Singapore"},{"name":"Duke\u2010NUS Medical School Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Puay H","family":"Tan","sequence":"additional","affiliation":[{"name":"Department of Anatomical Pathology Singapore General Hospital Singapore"},{"name":"Duke\u2010NUS Medical School Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bin T","family":"Teh","sequence":"additional","affiliation":[{"name":"Laboratory of Cancer Epigenome National Cancer Centre Singapore"},{"name":"Duke\u2010NUS Medical School Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth T E","family":"Chang","sequence":"additional","affiliation":[{"name":"VIVA\u2010KKH Paediatric Brain and Solid Tumour Laboratory KK Women's and Children's Hospital Singapore"},{"name":"Department of Pathology and Laboratory Medicine KK Women's and Children's Hospital Singapore"},{"name":"Duke\u2010NUS Medical School Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,10,30]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200001000-00002"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.4040"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.3985"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.18632\/oncotarget.5882"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.18632\/oncotarget.4682"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.canlet.2014.11.057"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.22316"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms9891"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.3338"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.18632\/oncotarget.7152"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.4693"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e31824a7b1a"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1115528109"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2012.04.009"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2001.19.2.488"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(197810)42:4<1922::AID-CNCR2820420434>3.0.CO;2-Z"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2013.06.036"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMp048113"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.2350\/14-12-1581-OA.1"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.12641"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000697"},{"key":"e_1_2_8_23_1","volume-title":"WHO classification of tumours of the urinary system and male genital organs","author":"Moch H","year":"2016"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btp324"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btv098"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btp352"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1038\/nbt.1754"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btq033"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkq603"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1186\/gb-2013-14-4-r36"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1186\/gb-2009-10-3-r25"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1101\/gr.229202"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/bts635"},{"key":"e_1_2_8_34_1","first-page":"511A","article-title":"Fusion gene detection in sarcomas by the NanoString nCounter fusion gene analysis assay","volume":"96","author":"Chang KTE","year":"2016","journal-title":"Lab. Invest."},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.1107"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.25210"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2014.139"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cancergencyto.2004.02.024"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.5858\/2007-131-446-RTADQI"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.22320"},{"key":"e_1_2_8_41_1","volume-title":"WHO classification of tumours of soft tissue and bone","author":"Fletcher CDM","year":"2013"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000223"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1111\/pin.12319"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1177\/1093526617698263"},{"key":"e_1_2_8_45_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-05-092510"},{"key":"e_1_2_8_46_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-03-272591"},{"key":"e_1_2_8_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cancergen.2014.09.001"},{"key":"e_1_2_8_48_1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1080\/10428194.2017.1283031","article-title":"Genomic instability is a principle pathologic feature of FLT3 ITD kinase activity in acute myeloid leukemia leading to clonal evolution and disease progression","volume":"58","author":"Rebechi MT","year":"2017","journal-title":"Leuk. Lymphoma"},{"key":"e_1_2_8_49_1","doi-asserted-by":"crossref","DOI":"10.1002\/pbc.26602","article-title":"Clear cell sarcoma of kidney involving a horseshoe kidney and harboring EGFR internal tandem duplication","volume":"64","author":"Santiago T","year":"2017","journal-title":"Pediatr. Blood Cancer"},{"key":"e_1_2_8_50_1","first-page":"1911","article-title":"Internal tandem duplication of the flt3 gene found in acute myeloid leukemia","volume":"10","author":"Nakao M","year":"1996","journal-title":"Leukemia"},{"key":"e_1_2_8_51_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2400812"},{"key":"e_1_2_8_52_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.onc.1205332"},{"key":"e_1_2_8_53_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000629"},{"key":"e_1_2_8_54_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2017.42"},{"key":"e_1_2_8_55_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2016.01.015"},{"key":"e_1_2_8_56_1","doi-asserted-by":"publisher","DOI":"10.1101\/gad.14.14.1810"},{"key":"e_1_2_8_57_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.str.2013.02.013"}],"container-title":["Histopathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhis.13366","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/his.13366","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,30]],"date-time":"2023-09-30T03:45:02Z","timestamp":1696045502000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/his.13366"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,10,30]]},"references-count":56,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2018,1]]}},"alternative-id":["10.1111\/his.13366"],"URL":"https:\/\/doi.org\/10.1111\/his.13366","archive":["Portico"],"relation":{},"ISSN":["0309-0167","1365-2559"],"issn-type":[{"value":"0309-0167","type":"print"},{"value":"1365-2559","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,10,30]]},"assertion":[{"value":"2017-07-03","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2017-08-16","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2017-10-30","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}