^
    3d
    Asymptomatic giant primary Ewing sarcoma of the skull in an adolescent: a case report and review of the literature. (PubMed, Childs Nerv Syst)
    This case underscores the importance of a multimodal approach in managing primary cranial Ewing's sarcoma. It emphasizes the need for further studies to evaluate its unique biological behavior and optimal treatment strategies.
    Review • Journal
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    VIM (Vimentin) • CD99 (CD99 Molecule)
    4d
    Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders (The Pediatric MATCH Screening Trial) (clinicaltrials.gov)
    P2, N=1376, Active, not recruiting, National Cancer Institute (NCI) | Trial completion date: May 2026 --> Jan 2027
    Trial completion date
    |
    BRAF (B-raf proto-oncogene)
    |
    Lynparza (olaparib) • Ibrance (palbociclib) • Zelboraf (vemurafenib) • Vitrakvi (larotrectinib) • Koselugo (selumetinib) • Balversa (erdafitinib) • Retevmo (selpercatinib) • Ensacove (ensartinib) • Zarnestra (tipifarnib) • Tibsovo (ivosidenib) • Tazverik (tazemetostat) • ulixertinib (BVD-523) • samotolisib (LY3023414)
    4d
    Primary Cutaneous Ewing Sarcoma With Unusual Features: Potential Mimic of Merkel Cell Carcinoma. (PubMed, Am J Dermatopathol)
    It underscores that dot-like CK20 and Neurofilament staining are not entirely pathognomonic for MCC diagnosis. In the differential diagnosis of cutaneous round cell tumors, which includes metastases, melanoma, and several sarcoma types, the present case reveals the diagnostic challenges of cutaneous sarcomas and highlights the necessity of a multimodal approach for accurate diagnosis.
    Journal
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    EWSR1 (EWS RNA Binding Protein 1) • SOX10 (SRY-Box 10) • FLI1 (Fli-1 Proto-Oncogene ETS Transcription Factor) • CD99 (CD99 Molecule)
    4d
    CD99Targeted Irinotecan Containing Nanoparticles Show Twenty-Fold Greater Anti-Tumor Effect Than Free Irinotecan For Treatment of Ewing Sarcoma. (PubMed, bioRxiv)
    NV103 was compared with free irinotecan, untargeted nanoparticles, and Onivyde TM at multiple dosages...These findings suggest that NV103 is a promising translational therapeutic agent that enhances the therapeutic index of irinotecan and other cytotoxic agents. This platform offers a broadly adaptable approach to tumor-specific drug delivery, which could significantly improve treatment outcomes and reduce long-term toxicity in children and young adults with Ewing sarcoma and other solid tumors.
    Journal
    |
    CD99 (CD99 Molecule)
    |
    Onivyde (nanoliposomal irinotecan)
    4d
    Validation of a seven-gene predictive SIGNature for the efficacy of Immuno-Oncology PD-1 inhibitors in patients with Sarcoma (SIGNIOS). (PubMed, Clin Cancer Res)
    This external validation confirms the seven-gene signature as a potential biomarker for predicting ICI efficacy in advanced sarcomas. Prospective studies are needed to determine the prognostic versus predictive value of SIGNIOS, refine its use across sarcoma subtypes, and explore its applicability in other tumor types.
    Journal • PD(L)-1 Biomarker • IO biomarker
    |
    LAG3 (Lymphocyte Activating 3) • CXCL10 (Chemokine (C-X-C motif) ligand 10) • CXCL9 (Chemokine (C-X-C motif) ligand 9) • CHI3L1 (Chitinase 3-like 1) • NR4A1 (Nuclear Receptor Subfamily 4 Group A Member 1) • VCAM1 (Vascular Cell Adhesion Molecule 1) • CD86 (CD86 Molecule)
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    Keytruda (pembrolizumab) • pazopanib
    5d
    Pathway for the Development of ATR Inhibitors in Pediatric Malignancies: An ACCELERATE Multistakeholder Analysis. (PubMed, JCO Precis Oncol)
    ATR inhibitors are a prototype for the development of medicinal products in a limited pediatric population. For the substantial potential of ATR inhibitors in children with malignancy to be realized, strategic planning between academia, industry, regulators, and patient advocates is vital.
    Review • Journal
    |
    MYCN (MYCN Proto-Oncogene BHLH Transcription Factor) • AURKA (Aurora kinase A)
    |
    MYCN amplification
    5d
    Current therapies in Ewing sarcoma: What's new? (PubMed, Bull Cancer)
    Immunotherapeutic approaches are also being investigated, particularly CAR-T and CAR-NK cell therapy. Close collaboration between clinicians and biologists has also highlighted the importance of biomarkers that are still being validated prospectively and might be incorporated into standard of care in the future.
    Review • Journal • IO biomarker
    |
    EWSR1 (EWS RNA Binding Protein 1) • FLI1 (Fli-1 Proto-Oncogene ETS Transcription Factor)
    5d
    HER2 Chimeric Antigen Receptor (CAR) T Cells in Combination With Checkpoint Blockade in Patients With Advanced Sarcoma (clinicaltrials.gov)
    P1, N=25, Recruiting, Baylor College of Medicine | Trial completion date: Dec 2040 --> Jun 2043 | Trial primary completion date: Dec 2027 --> Jun 2028
    Trial completion date • Trial primary completion date • Checkpoint inhibition
    |
    HER-2 (Human epidermal growth factor receptor 2)
    |
    HER-2 positive • HER-2 expression
    |
    Keytruda (pembrolizumab) • Opdivo (nivolumab)
    6d
    A Clinical Study of TK216 in Patients With Relapsed or Refractory Ewing's Sarcoma (clinicaltrials.gov)
    P2, N=30, Recruiting, Shanghai Pharmaceuticals Holding Co., Ltd | Trial completion date: Dec 2025 --> Dec 2026 | Trial primary completion date: Sep 2025 --> Sep 2026
    Trial completion date • Trial primary completion date
    |
    ONCT-216
    8d
    Fe3O4 nanozyme integrated upconversion luminescence synergistic energy transfer composite nanoplatform for the ultrasensitive detection of proGRP. (PubMed, Anal Chim Acta)
    This work presents an innovative sensing platform that combines near-infrared light-excited luminescence with nanozyme-mediated signal amplification for the detection of proGRP. The method surpasses conventional assays in sensitivity and operational stability, showing great promise for clinical translation. Its robust performance in serum underscores potential applications in early cancer diagnostics and biomarker-based disease management, providing a valuable tool for disease diagnosis.
    Journal
    |
    DNA2 (DNA Replication Helicase/Nuclease 2)
    10d
    Metronomic 5-Fluorouracil and Vinorelbine Reduce Cancer Stemness and Modulate EZH2/NOTCH-1/STAT3 Signaling in Triple-Negative Breast Cancer Spheroids. (PubMed, Int J Mol Sci)
    Unlike STD, which partially and transiently reduced stemness markers, mCHT achieved sustained suppression, indicating preferential targeting of therapy-resistant CSCs. These results indicate mCHT as a promising strategy for specifically aiming at the CSC-like compartment in TNBC, underscoring a therapeutic approach that reprograms key epigenetic networks and overcomes resistance to treatment.
    Journal
    |
    NOTCH1 (Notch 1) • STAT3 (Signal Transducer And Activator Of Transcription 3) • CD44 (CD44 Molecule)
    |
    5-fluorouracil • vinorelbine tartrate
    10d
    Genomic Profiling of Highly Aggressive Musculoskeletal Sarcomas Identifies Potential Therapeutic Targets: A Single-Center Experience. (PubMed, Cancers (Basel))
    Early access to genomic analyses, routine germline assessment, and broad gene panels would help in identifying possible targeted drugs with sufficient evidence of activity beneficial to each patient. In the clinical management of advanced sarcoma patients, when analyzing cost-effectiveness and sustainability, the role of the Molecular Tumor Board in the governance of the complexity introduced by mutational oncology should be considered.
    Journal
    |
    TP53 (Tumor protein P53) • PTEN (Phosphatase and tensin homolog) • ROS1 (Proto-Oncogene Tyrosine-Protein Kinase ROS) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • ARID1A (AT-rich interaction domain 1A) • AKT1 (V-akt murine thymoma viral oncogene homolog 1) • SMARCA4 (SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily A, member 4) • CDKN2B (Cyclin Dependent Kinase Inhibitor 2B) • PMS2 (PMS1 protein homolog 2) • MUC16 (Mucin 16, Cell Surface Associated) • NOTCH4 (Notch 4) • RHOA (Ras homolog family member A) • BARD1 (BRCA1 Associated RING Domain 1) • CCND3 (Cyclin D3) • DDR2 (Discoidin domain receptor 2) • BMPR1A (Bone Morphogenetic Protein Receptor Type 1A)
    |
    Archer® VariantPlex® Solid Tumor Kit