{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T20:23:49Z","timestamp":1787775829692,"version":"build-2784847793"},"reference-count":15,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","license":[{"start":{"date-parts":[[2024,9,14]],"date-time":"2024-09-14T00:00:00Z","timestamp":1726272000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2024,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    <jats:italic toggle=\"yes\">BCOR-<\/jats:italic>\n                    rearranged sarcomas (BRS) constitute relatively newly described sarcomas, which, within the musculoskeletal sites, usually occur in the bones, followed by soft tissues. Primary BRS involving the visceral organs is very rare, and only a single case is reported in the lung. These tumors share overlapping morphological and immunohistochemical (IHC) features with other neoplasms, such as synovial sarcoma, Ewing sarcoma, as well as carcinosarcoma, the latter especially when occurring in the visceral organs. BCOR immunostaining is useful in triaging a tumor for molecular diagnosis, which constitutes the \u201cessential\u201d diagnostic criterion for these tumors. To report an extremely rare case of a BRS, confirmed by\n                    <jats:italic toggle=\"yes\">BCOR<\/jats:italic>\n                    -rearrangement by fluorescence\n                    <jats:italic toggle=\"yes\">in situ<\/jats:italic>\n                    hybridization (FISH), primarily occurring in the lung, emphasizing the diagnostic approach and management, along with review of literature. An18-year-old boy presented with complaints of left-sided chest pain, along with cough, fever, loss of appetite, and weight. On radio imaging, there was a complete collapse of the left lower lobe of lung with moderate pleural effusion. The biopsy showed a biphasic tumor comprising primitive round cells admixed with spindle cells. Immunohistochemically, the tumor cells were positive for BCOR, TLE1, and p53. FISH showed\n                    <jats:italic toggle=\"yes\">BCOR<\/jats:italic>\n                    gene rearrangement. A diagnosis of primary BRS of lung was offered. The patient had a favorable response to the chemotherapy regime. BRS is an ultra-rare tumor, which rarely involves visceral organs. The lung is an exceptionally rare site, with only single reported case previously. An exact confirmation by molecular testing has treatment-associated implications. A review of similar reported cases is presented herewith.\n                  <\/jats:p>","DOI":"10.4103\/ijpm.ijpm_569_24","type":"journal-article","created":{"date-parts":[[2024,9,17]],"date-time":"2024-09-17T13:18:04Z","timestamp":1726579084000},"page":"865-869","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":1,"title":["The BCOR-rearranged sarcoma involving the lung: Diagnosis with clinical outcome and literature review"],"prefix":"10.4103","volume":"67","author":[{"given":"Rakesh K.","family":"Gupta","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bharat","family":"Rekhi","sequence":"additional","affiliation":[{"name":"Department of Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mehar C.","family":"Sharma","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ranganath","family":"Ganga","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Medicine, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mudalsha","family":"Ravina","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amit","family":"Kumar","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dibakar","family":"Sahu","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Medicine, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2024,9,14]]},"reference":[{"key":"R1-20241206","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1038\/ng.1107","article-title":"A new subtype of bone sarcoma defined by BCOR-CCNB3 gene fusion","volume":"44","author":"Pierron","year":"2012","journal-title":"Nat Genet"},{"key":"R2-20241206","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1016\/j.molcel.2012.01.002","article-title":"PCGF homologs, CBX proteins, and RYBP define functionally distinct PRC1 family complexes","volume":"45","author":"Gao","year":"2012","journal-title":"Mol Cell"},{"key":"R3-20241206","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1097\/PAS.0000000000000223","article-title":"BCOR-CCNB3 (Ewing-like) sarcoma:A clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma","volume":"38","author":"Puls","year":"2014","journal-title":"Am J Surg Pathol"},{"key":"R4-20241206","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1111\/his.14023","article-title":"Expanding the clinicopathologic and molecular spectrum of BCOR-associated sarcomas in adults","volume":"76","author":"Yoshida","year":"2020","journal-title":"Histopathology"},{"key":"R5-20241206","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1038\/modpathol.2014.139","article-title":"BCOR-CCNB3 fusions are frequent in undifferentiated sarcomas of male children","volume":"28","author":"Peters","year":"2015","journal-title":"Mod Pathol"},{"key":"R6-20241206","first-page":"e59731","article-title":"A report of a rare case of BCOR (B-cell Line 6 Corepressor)-altered sarcoma of the lung in a child","volume":"16","author":"Capellan","year":"2024","journal-title":"Cureus"},{"key":"R7-20241206","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1097\/PAS.0000000000000965","article-title":"BCOR-CCNB3 fusion positive sarcomas:A clinicopathologic and molecular analysis of 36 cases with comparison to morphologic spectrum and clinical behavior of other round cell sarcomas","volume":"42","author":"Kao","year":"2018","journal-title":"Am J Surg Pathol"},{"key":"R8-20241206","doi-asserted-by":"crossref","first-page":"1702","DOI":"10.1097\/PAS.0000000000000926","article-title":"Primary renal sarcomas with BCOR-CCNB3 gene fusion:A report of 2 cases showing histologic overlap with clear cell sarcoma of kidney, suggesting further link between BCOR-related sarcomas of the kidney and soft tissues","volume":"41","author":"Argani","year":"2017","journal-title":"Am J Surg Pathol"},{"key":"R9-20241206","doi-asserted-by":"crossref","first-page":"105290","DOI":"10.1016\/j.oraloncology.2021.105290","article-title":"BCOR-CCNB3 rearranged sarcoma arising in neck misdiagnosed as thyroid cancer:A case report","volume":"120","author":"Tan","year":"2021","journal-title":"Oral Oncol"},{"key":"R10-20241206","first-page":"1455613221141612","article-title":"BCOR-Rearranged sarcoma of the tonsil","author":"Bosco","year":"2023","journal-title":"Ear Nose Throat J"},{"key":"R11-20241206","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1016\/j.anl.2022.04.012","article-title":"BCOR-CCNB3 sarcoma arising in the pharynx","volume":"50","author":"Eguchi","year":"2023","journal-title":"Auris Nasus Larynx"},{"key":"R12-20241206","doi-asserted-by":"crossref","first-page":"1670","DOI":"10.1097\/PAS.0000000000000697","article-title":"BCOR overexpression is a highly sensitive marker in round cell sarcomas with BCOR genetic abnormalities","volume":"40","author":"Kao","year":"2016","journal-title":"Am J Surg Pathol"},{"key":"R13-20241206","doi-asserted-by":"crossref","first-page":"557","DOI":"10.4103\/ijmr.IJMR_2144_18","article-title":"Clinicopathologic features of undifferentiated round cell sarcomas of bone and soft tissues:An attempt to unravel the BCOR-CCNB3- and CIC-DUX4-positive sarcomas","volume":"150","author":"Rekhi","year":"2019","journal-title":"Indian J Med Res"},{"key":"R14-20241206","doi-asserted-by":"crossref","first-page":"941","DOI":"10.1097\/PAS.0000000000000846","article-title":"Sarcomas with CIC-rearrangements are a distinct pathologic entity with aggressive outcome:A clinicopathologic and molecular study of 115 cases","volume":"41","author":"Antonescu","year":"2017","journal-title":"Am J Surg Pathol"},{"key":"R15-20241206","doi-asserted-by":"crossref","first-page":"1244","DOI":"10.1177\/10668969221143467","article-title":"Spectrum of histopathological, immunohistochemical, molecular and radiological features in 12 cases of BCOR:CCNB3-positive sarcomas with literature review","volume":"31","author":"Rekhi","year":"2023","journal-title":"Int J Surg Pathol"}],"container-title":["Indian Journal of Pathology and Microbiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/ijpm.ijpm_569_24","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T19:43:23Z","timestamp":1787773403000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/ijpm.ijpm_569_24"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9,14]]},"references-count":15,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2024]]}},"URL":"https:\/\/doi.org\/10.4103\/ijpm.ijpm_569_24","relation":{},"ISSN":["0377-4929","0974-5130"],"issn-type":[{"value":"0377-4929","type":"print"},{"value":"0974-5130","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,9,14]]},"assertion":[{"value":"2024-07-08","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-08-16","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}