{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,21]],"date-time":"2025-11-21T07:34:48Z","timestamp":1763710488072,"version":"3.45.0"},"reference-count":12,"publisher":"Scientific Scholar","content-domain":{"domain":["surgicalneurologyint.com"],"crossmark-restriction":true},"short-container-title":[],"abstract":"\n Background:<\/jats:title>\n Central nervous system (CNS) high-grade neuroepithelial tumors with BCL-6 co-repressor (BCOR) exon 15 internal tandem-duplication (ITD) are rare, aggressive pediatric neoplasms characterized by poor prognosis and unclear treatment protocols. The diagnosis requires molecular confirmation, and early neurosurgical intervention is critical.<\/jats:p>\n <\/jats:sec>\n \n Case Description:<\/jats:title>\n A 2-year-old male presented with progressive fatigue, ataxia, and signs of increased intracranial pressure. Imaging revealed a large left frontal mass with midline shift. Emergent bifrontal craniotomy was performed, achieving gross total resection (GTR) without preoperative MRI due to hemodynamic instability. Subsequent deoxyribonucleic acid methylation profiling confirmed the diagnosis of a CNS BCOR-ITD. The patient was treated with intensive chemotherapy following the ACNS0334 protocol, followed by autologous hematopoietic stem cell transplantation. He remains neurologically intact at the past follow-up 9 months after surgery.<\/jats:p>\n <\/jats:sec>\n \n Conclusion:<\/jats:title>\n This case highlights the diagnostic and therapeutic challenges of pediatric CNS BCOR-ITD, emphasizing the need to attempt early GTR and multimodal chemotherapy in providing a meaningful functional recovery for this patient.<\/jats:p>\n <\/jats:sec>","DOI":"10.25259\/sni_439_2025","type":"journal-article","created":{"date-parts":[[2025,11,21]],"date-time":"2025-11-21T07:29:48Z","timestamp":1763710188000},"page":"489","update-policy":"https:\/\/doi.org\/10.25259\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Pediatric high-grade neuroepithelial BCOR tandem duplicated tumor: An illustrative case"],"prefix":"10.25259","volume":"16","author":[{"given":"Nicholas Edward","family":"Bui","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey","family":"Lubisich","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Diem Kieu","family":"Tran","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"10894","published-online":{"date-parts":[[2025,11,21]]},"reference":[{"key":"10.25259\/SNI_439_2025\/ref-1","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1002\/cjp2.255","article-title":"Intra- and extra-cranial BCOR-ITD tumours are separate entities within the BCOR -rearranged family","volume":"8","author":"Bouchoucha","year":"2022","journal-title":"J Pathol Clin Res"},{"key":"10.25259\/SNI_439_2025\/ref-2","doi-asserted-by":"crossref","first-page":"151","DOI":"10.3174\/ajnr.A7367","article-title":"Imaging features with histopathologic correlation of CNS high-grade neuroepithelial tumors with a BCOR internal tandem duplication","volume":"43","author":"Cardoen","year":"2022","journal-title":"AJNR Am J Neuroradiol"},{"key":"10.25259\/SNI_439_2025\/ref-3","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1111\/bpa.12747","article-title":"High-grade neuroepithelial tumor with BCOR exon 15 internal tandem duplication-a comprehensive clinical, radiographic, pathologic, and genomic analysis","volume":"30","author":"Ferris","year":"2020","journal-title":"Brain Pathol"},{"key":"10.25259\/SNI_439_2025\/ref-4","doi-asserted-by":"crossref","first-page":"1161","DOI":"10.1101\/gad.337584.120","article-title":"Functional loss of a noncanonical BCORPRC1.1 complex accelerates SHH-driven medulloblastoma formation","volume":"34","author":"Kutscher","year":"2020","journal-title":"Genes Dev"},{"key":"10.25259\/SNI_439_2025\/ref-5","doi-asserted-by":"crossref","first-page":"1231","DOI":"10.1093\/neuonc\/noab106","article-title":"The 2021 WHO Classification of tumors of the central nervous system: A summary","volume":"23","author":"Louis","year":"2021","journal-title":"Neuro Oncol"},{"key":"10.25259\/SNI_439_2025\/ref-6","doi-asserted-by":"crossref","first-page":"114210","DOI":"10.18632\/oncotarget.23174","article-title":"Personalized therapy: CNS HGNET-BCOR responsiveness to arsenic trioxide combined with radiotherapy","volume":"8","author":"Paret","year":"2017","journal-title":"Oncotarget"},{"key":"10.25259\/SNI_439_2025\/ref-7","doi-asserted-by":"crossref","first-page":"83378","DOI":"10.18632\/oncotarget.13092","article-title":"Activation of the basal cell carcinoma pathway in a patient with CNS HGNET-BCOR diagnosis: Consequences for personalized targeted therapy","volume":"7","author":"Paret","year":"2016","journal-title":"Oncotarget"},{"key":"10.25259\/SNI_439_2025\/ref-8","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1038\/nature11329","article-title":"Medulloblastoma exome sequencing uncovers subtype-specific somatic mutations","volume":"488","author":"Pugh","year":"2012","journal-title":"Nature"},{"key":"10.25259\/SNI_439_2025\/ref-9","doi-asserted-by":"crossref","first-page":"1060","DOI":"10.1016\/j.cell.2016.01.015","article-title":"New brain tumor entities emerge from molecular classification of CNS-PNETs","volume":"164","author":"Sturm","year":"2016","journal-title":"Cell"},{"key":"10.25259\/SNI_439_2025\/ref-10","doi-asserted-by":"crossref","first-page":"153995","DOI":"10.1016\/j.prp.2022.153995","article-title":"CNS tumor with BCOR internal tandem duplication: Clinicopathologic, molecular characteristics and prognosis factors","volume":"236","author":"Wang","year":"2022","journal-title":"Pathol Res Pract"},{"key":"10.25259\/SNI_439_2025\/ref-11","doi-asserted-by":"crossref","first-page":"20190237","DOI":"10.1259\/bjr.20190237","article-title":"Proton therapy for brain tumours in the area of evidence-based medicine","volume":"93","author":"Weber","year":"2020","journal-title":"Br J Radiol"},{"key":"10.25259\/SNI_439_2025\/ref-12","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1111\/bpa.12585","article-title":"CNS high-grade neuroepithelial tumor with BCOR internal tandem duplication: A comparison with its counterparts in the kidney and soft tissue","volume":"28","author":"Yoshida","year":"2017","journal-title":"Brain Pathol"}],"container-title":["Surgical Neurology International"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/surgicalneurologyint.com\/pediatric-high-grade-neuroepithelial-bcor-tandem-duplicated-tumor-an-illustrative-case\/","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/surgicalneurologyint.com\/surgicalint-articles\/pediatric-high-grade-neuroepithelial-bcor-tandem-duplicated-tumor-an-illustrative-case\/","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,21]],"date-time":"2025-11-21T07:29:50Z","timestamp":1763710190000},"score":1,"resource":{"primary":{"URL":"http:\/\/surgicalneurologyint.com\/surgicalint-articles\/pediatric-high-grade-neuroepithelial-bcor-tandem-duplicated-tumor-an-illustrative-case\/"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,21]]},"references-count":12,"alternative-id":["10.25259\/SNI_439_2025"],"URL":"https:\/\/doi.org\/10.25259\/sni_439_2025","relation":{},"ISSN":["2152-7806","2229-5097"],"issn-type":[{"value":"2152-7806","type":"electronic"},{"value":"2229-5097","type":"print"}],"subject":[],"published":{"date-parts":[[2025,11,21]]},"article-number":"489"}}