ArticleCancer research2024
Comprehensive Transcriptomic Analysis of EWSR1::WT1 Targets Identifies CDK4/6 Inhibitors as an Effective Therapy for Desmoplastic Small Round Cell Tumors.
Article in Cancer research, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
17 citing papers in PubMed, 16 citations in OpenAlex.
- Review
- Desmoplastic Small Round Blue Cell Tumor in a Young Adult Man: A Rare Case of Abdominal Sarcoma.The American journal of case reports · 2026Article
- The emerging role of fatty acids as key bioactive components of Ganoderma lucidum spore oil in alleviating tumor progression.Journal of natural medicines · 2026Review
- Intracranial Mesenchymal Tumor with FET-CREB Gene Fusion: A Case Report of Late Recurrence.OncoTargets and therapy · 2026Article
- Case Report: Refractory desmoplastic small round cell tumor: a report of a molecularly confirmed case withFrontiers in oncology · 2026Article
- An Autopsy Case of a Desmoplastic Small Round Cell Tumor with a Rapid Fatal Outcome due to Diffuse Cystic Liver Metastases.Internal medicine (Tokyo, Japan) · 2025Article
- WT1 gene: a potential therapeutic target for multiple cancer treatment strategies.Clinical and experimental medicine · 2025Review
- EWS::WT1 Isoform-Dependent Regulation of Neogenes in Desmoplastic Small Round Cell Tumors.bioRxiv : the preprint server for biology · 2025Article
- Desmoplastic Small Round Cell Tumors and the Role of Androgen Receptors.Current treatment options in oncology · 2025Review
- Gene Fusions as Potential Therapeutic Targets in Soft Tissue Sarcomas.Biomolecules · 2025Review
- Comprehensive DSRCT multi-omics analyses unveil CACNA2D2 as a diagnostic hallmark and super-enhancer-driven EWSR1::WT1 signature gene.Cancer communications (London, England) · 2025Article
- Desmoplastic small round cell tumor: an update of current management practices.Journal of the Egyptian National Cancer Institute · 2025Review
- Primary desmoplastic small round cell tumour of the prostate.Journal of clinical pathology · 2024Article
- Review
- HER2 Antibody-Drug Conjugates Are Active against Desmoplastic Small Round Cell Tumor.Clinical cancer research : an official journal of the American Association for Cancer Research · 2024Article
- Desmoplastic small round cell tumor: from genomics to targets, potential paths to future therapeutics.Frontiers in cell and developmental biology · 2024Review
- Unresectable Desmoplastic Small Round Cell Tumor: A Case Report and Review of the Literature.Case reports in oncologyArticle
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
Abstract
Desmoplastic small round cell tumors (DSRCT) are a type of aggressive, pediatric sarcoma characterized by the EWSR1::WT1 fusion oncogene. Targeted therapies for DSRCT have not been developed, and standard multimodal therapy is insufficient, leading to a 5-year survival rate of only 15% to 25%. Here, we depleted EWSR1::WT1 in DSRCT and established its essentiality in vivo. Transcriptomic analysis revealed that EWSR1::WT1 induces unique transcriptional alterations compared with WT1 and other fusion oncoproteins and that EWSR1::WT1 binding directly mediates gene upregulation. The E-KTS isoform of EWSR1::WT1 played a dominant role in transcription, and it bound to the CCND1 promoter and stimulated DSRCT growth through the cyclin D-CDK4/6-RB axis. Treatment with the CDK4/6 inhibitor palbociclib successfully reduced growth in two DSRCT xenograft models. As palbociclib has been approved by the FDA for the treatment of breast cancer, these findings demonstrate the sensitivity of DSRCT to palbociclib and support immediate clinical investigation of palbociclib for treating this aggressive pediatric cancer. SIGNIFICANCE: EWSR1::WT1 is essential for desmoplastic small round cell tumors and upregulates the cyclin D-CDK4/6-RB axis that can be targeted with palbociclib, providing a targeted therapeutic strategy for treating this deadly tumor type.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.