ArticleBMC cancer2014
Establishment of a novel clear cell sarcoma cell line (Hewga-CCS), and investigation of the antitumor effects of pazopanib on Hewga-CCS.
Article in BMC cancer, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 33 citations in OpenAlex.
- Phase II trial of pazopanib in patients with metastatic or unresectable chemoresistant sarcomas: A Japanese Musculoskeletal Oncology Group study.Cancer science · 2020Trial
- Cooperative Roles of Class IA PI3K Isoforms in Translocation-Related Sarcoma Cell Survival and Proliferation.Cancer research communications · 2026Article
- Establishing preclinical models for clear cell sarcoma of soft tissue.Frontiers in oncology · 2025Article
- Prognostic factors in clear cell sarcoma: an analysis of soft tissue sarcoma in 43 cases.Journal of cancer research and clinical oncology · 2024Article
- Biological and therapeutic insights from animal modeling of fusion-driven pediatric soft tissue sarcomas.Disease models & mechanisms · 2024Review
- Functional genomics of human clear cell sarcoma: genomic, transcriptomic and chemical biology landscape for clear cell sarcoma.British journal of cancer · 2023Article
- Targeting epigenetic features in clear cell sarcomas based on patient-derived cell lines.Journal of translational medicine · 2023Article
- Activity of pazopanib inOncoscience · 2023Article
- Establishment of Organoids From Human Epithelioid Sarcoma With the Air-Liquid Interface Organoid Cultures.Frontiers in oncology · 2022Article
- Genomics, Morphoproteomics, and Treatment Patterns of Patients with Alveolar Soft Part Sarcoma and Response to Multiple Experimental Therapies.Molecular cancer therapeutics · 2020Article
- Establishment of a novel human CIC-DUXScientific reports · 2019Article
- Primary clear cell sarcoma of the calcaneus : Report of a rare case and review of the literature.Der Orthopade · 2019Review
- Characterization of pulmonary intimal sarcoma cells isolated from a surgical specimen: In vitro and in vivo study.PloS one · 2019Article
- Establishment and proteomic characterization of patient-derived clear cell sarcoma xenografts and cell lines.In vitro cellular & developmental biology. Animal · 2018Article
- Trabectedin is a promising antitumor agent potentially inducing melanocytic differentiation for clear cell sarcoma.Cancer medicine · 2017Article
- Treatment of advanced, metastatic soft tissue sarcoma: latest evidence and clinical considerations.Therapeutic advances in medical oncology · 2017Review
- Therapeutic potential of TAS-115 via c-MET and PDGFRα signal inhibition for synovial sarcoma.BMC cancer · 2017Article
- A retrospective cohort study of treatment patterns among patients with metastatic soft tissue sarcoma in the US.Clinical sarcoma research · 2017Article
- Functional and therapeutic relevance of hepatocyte growth factor/c-MET signaling in synovial sarcoma.Cancer science · 2016Article
Corrections and comments
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundClear cell sarcoma (CCS) is a therapeutically unresolved, aggressive, soft tissue sarcoma (STS) that predominantly affects young adults. This sarcoma is defined by t(12;22)(q13;q12) translocation, which leads to the fusion of Ewing sarcoma gene (EWS) to activating transcription factor 1 (ATF1) gene, producing a chimeric EWS-ATF1 fusion gene. We established a novel CCS cell line called Hewga-CCS and developed an orthotopic tumor xenograft model to enable comprehensive bench-side investigation for intensive basic and preclinical research in CCS with a paucity of experimental cell lines.
methodsHewga-CCS was derived from skin metastatic lesions of a CCS developed in a 34-year-old female. The karyotype and chimeric transcript were analyzed. Xenografts were established and characterized by morphology and immunohistochemical reactivity. Subsequently, the antitumor effects of pazopanib, a recently approved, novel, multitargeted, tyrosine kinase inhibitor (TKI) used for the treatment of advanced soft tissue sarcoma, on Hewga-CCS were assessed in vitro and in vivo.
resultsHewga-CCS harbored the type 2 EWS-ATF1 transcript. Xenografts morphologically mimicked the primary tumor and expressed S-100 protein and antigens associated with melanin synthesis (Melan-A, HMB45). Pazopanib suppressed the growth of Hewga-CCS both in vivo and in vitro. A phospho-receptor tyrosine kinase array revealed phosphorylation of c-MET, but not of VEGFR, in Hewga-CCS. Subsequent experiments showed that pazopanib exerted antitumor effects through the inhibition of HGF/c-MET signaling.
conclusionsCCS is a rare, devastating disease, and our established CCS cell line and xenograft model may be a useful tool for further in-depth investigation and understanding of the drug-sensitivity mechanism.
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