ArticleThe American journal of surgical pathology2017
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.
Article in The American journal of surgical pathology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 30 papers, 1 of them a synthesis that pooled it.
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Who cites it
30 citing papers in PubMed, 1 synthesis or guideline pooled it, 85 citations in OpenAlex.
- Primary Renal Synovial Sarcoma and Clinical and Pathological Findings: a Systematic Review.Current urology reports · 2021Pooled it
- ZC3H7A/B::BCOR fusion fibromyxoid sarcoma of soft tissue: an emerging aggressive sarcoma overlapping with malignant ossifying fibromyxoid tumors.Virchows Archiv : an international journal of pathology · 2025Article
- MEIS1::NCOA1 Primitive Spindle Cell Sarcoma of the Kidney : Report of 7 Cases of a Distinctive Clinicopathologic Entity.The American journal of surgical pathology · 2025Article
- BCOR abnormalities in endometrial stromal sarcoma.Gynecologic oncology reports · 2025Review
- Clinicopathologic and proteomic characteristics of low-grade undifferentiated spindle cell sarcoma.Frontiers in molecular biosciences · 2025Article
- Primary renal BCOR::CCNB3 sarcoma in a female patient: case report.Journal of pathology and translational medicine · 2025Article
- Synovial Sarcoma of the Kidney: Diagnostic Pitfalls in a Case with Myxoid Monophasic Differentiation and No Epithelial Biomarkers Expression.International journal of molecular sciences · 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
- Article
- Primary renal sarcoma with SS18::POU5F1 gene fusion.Genes, chromosomes & cancer · 2022Article
- Paediatric BCOR-associated sarcomas with a novel long spliced internal tandem duplication of BCOR exon 15.The journal of pathology. Clinical research · 2022Article
- BCOR-CCNB3 sarcoma with concurrent RNF213-SLC26A11 gene fusion: a rare sarcoma with altered histopathological features after chemotherapy.World journal of surgical oncology · 2022Article
- Clinicopathologic and molecular analysis of aCold Spring Harbor molecular case studies · 2022Article
- Unclassified low grade spindle cell sarcoma with storiform pattern characterized by recurrent novel EWSR1/FUS-NACC1 fusions.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2021Article
- Integrated diagnosis based on transcriptome analysis in suspected pediatric sarcomas.NPJ genomic medicine · 2021Article
- Small Round Blue Cell Sarcoma Other Than Ewing Sarcoma: What Should an Oncologist Know?Current treatment options in oncology · 2020Review
- Review
- [Rare childhood kidney tumors].Der Pathologe · 2019Review
- BCOR Overexpression in Renal Malignant Solitary Fibrous Tumors: A Close Mimic of Clear Cell Sarcoma of Kidney.The American journal of surgical pathology · 2019Article
- BCOR involvement in cancer.Epigenomics · 2019Review
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Authors and funding
8 authors at 6 institutions in 3 countries.
Funding
Abstract
We report 2 primary renal sarcomas demonstrating BCOR-CCNB3 gene fusions that have recently been identified in undifferentiated round cell sarcomas of bone and soft tissue. These neoplasms occurred in male children aged 11 and 12 years, and both were cystic as a result of entrapment and dilatation of native renal tubules. Both cases were composed of variably cellular bland spindle cells with fine chromatin set in myxoid stroma and separated by a branching capillary vasculature. Both neoplasms demonstrated immunoreactivity for BCOR, cyclin D1, TLE1, and SATB2 in the spindle neoplastic cells and negativity in the prominent capillary vasculature. One case was extensively cystic and had hypocellular areas that simulated cystic nephroma; this neoplasm recurred 3 years later as a solid, highly cellular spindle cell sarcoma in the abdominal cavity. The morphology and immunoprofile of these renal neoplasms was compared with a control group of other sarcomas with BCOR genetic abnormalities, including clear cell sarcoma of the kidney (CCSK), infantile undifferentiated round cell sarcomas of soft tissue/primitive myxoid mesenchymal tumor of infancy, and bone/soft tissue sarcomas with BCOR-CCNB3 gene fusion; along with primary renal synovial sarcoma. Our findings show that the renal sarcomas with BCOR-CCNB3 gene fusion overlap with CCSK. These results are in keeping with a "BCOR-alteration family" of renal and extrarenal neoplasms which includes CCSK and undifferentiated round cell sarcomas of soft tissue/primitive myxoid mesenchymal tumor of infancy (which typically harbor BCOR internal tandem duplication), and BCOR-CCNB3 sarcomas, all of which are primarily driven by BCOR overexpression and have overlapping (but not identical) clinicopathologic features.
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Registered trials
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