ArticleCancer medicine2023
Clinical characteristics and outcomes for children, adolescents and young adults with "CIC-fused" or "BCOR-rearranged" soft tissue sarcomas: A multi-institutional European retrospective analysis.
Article in Cancer medicine, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed.
- CIC-rearranged sarcoma of the arm presenting as a soft tissue mass with regional lymphadenopathy and tumor thrombus.Skeletal radiology · 2026Article
- Article
- Article
- Superficially located CIC::DUX4-rearranged sarcomas in children: insights from a long-term survival case series.Virchows Archiv : an international journal of pathology · 2026Article
- A Real-World Analysis of Outcomes in CIC-Rearranged Sarcomas: A Canadian Sarcoma Research and Clinical Collaboration (CanSaRCC) Study.Cancer medicine · 2026Article
- Case Report: Refractory life-threatening post-biopsy hemorrhage in a neonate successfully controlled with Ankaferd Blood Stopper.Frontiers in pediatrics · 2026Article
- Review
- Patient-derived tumoroids from CIC::DUX4 rearranged sarcoma identify MCL1 as a therapeutic target.Nature communications · 2025Article
- CIC/ATXN1-rearranged tumors in the central nervous system are mainly represented by sarcomas: A comprehensive clinicopathological and epigenetic series.Brain pathology (Zurich, Switzerland) · 2025Article
- Infantile undifferentiated sarcomas: a diagnostic and therapeutic challenge - two case reports and literature review.Acta oncologica (Stockholm, Sweden) · 2025Review
- Nuclear DUX4 immunohistochemistry is a highly sensitive and specific marker for the presence of CIC::DUX4 fusion in CIC-rearranged sarcomas: a study of 48 molecularly confirmed cases.Histopathology · 2025Article
- A Report of a Rare Case of BCOR (B-cell Line 6 Corepressor)-Altered Sarcoma of the Lung in a Child.Cureus · 2024Article
- Patterns of expression of VEGFR2, PDGFRs and c-Kit in pediatric patients with high grade non-rhabdomyosarcoma soft tissue sarcoma.Frontiers in oncology · 2024Article
- Clinical characteristics and outcomes for children, adolescents and young adults with "CIC-fused" or "BCOR-rearranged" soft tissue sarcomas: A multi-institutional European retrospective analysis.Cancer medicine · 2023Article
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Abstract
backgroundIn certain rare undifferentiated small round cell sarcomas new specific molecular CIC-DUX4/other partner, BCOR-CCNB3/other partner, YWHAE fusions, or BCOR-ITD (internal tandem duplication) were identified. These new "CIC fused" (CIC-fused/ATXN1::NUTM1) and "BCOR rearranged" (BCOR fused/ITD/ YWHAE) soft tissue sarcomas (STS) are not well described.
methodsMulti-institutional European retrospective analysis of young patients (0-24 years) with CIC-fused and BCOR rearranged STS.
resultsOverall, out of the 60 patients selected, the fusion status was CIC-fused (n = 29), ATXN1::NUTM1 (n = 2), BCOR::CCNB3 (n = 18), BCOR-ITD (n = 7), and YWHAE (n = 3), MAML::BCOR STS (n = 1). The main primaries were abdomen-pelvic (n = 23) and limbs (n = 18). Median age was 14 years (0.9-23.8) and 0.9 (0.1-19.1) for CIC-fused and BCOR-rearranged groups, respectively (n = 29; p < 0.001). IRS stages were I (n = 3), II (n = 7), III (n = 35), and IV (n = 15). Overall, 42 patients had large tumors (>5 cm) but only six had lymph node involvement. Patients received mainly chemotherapy (n = 57), local surgery (n = 50), and/or radiotherapy (n = 34). After a median follow-up of 47.1 months (range, 3.4-230), 33 (52%) patients had an event and 23 patients died. Three-year event-free survivals were 44.0% (95% CI 28.7-67.5) and 41.2% (95% CI 25.4-67.0) for CIC and BCOR groups (p = 0.97), respectively. Three-year overall survivals were 46.3% (95% CI 29.6-72.4) and 67.1% (95% CI 50.4-89.3; p = 0.24), respectively.
conclusionsPediatric patients often present with large tumors and metastatic disease, especially CIC sarcomas. Overall outcome is dismal. New treatment options are needed.
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