^
    3d
    NTRK1-3 fusions in sarcomas: prevalence, significance, and clinical implications - a systematic review. (PubMed, Future Oncol)
    Larotrectinib was used in 142 patients, demonstrating an 83.80% response rate, with low mortality (2.82%) and recurrence (2.11%) rates. Entrectinib had a lower response rate of 63.64%. We confirm the rarity of NTRK1-3 fusions in sarcomas. TRK inhibitors show high efficacy in sarcomas, emphasizing the necessity of genomic testing in all cases.Protocol registration: https://www.crd.york.ac.uk/PROSPERO/view/CRD42024563594.
    Review • Journal
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    NTRK1 (Neurotrophic tyrosine kinase, receptor, type 1) • NTRK3 (Neurotrophic tyrosine kinase, receptor, type 3) • NTRK2 (Neurotrophic tyrosine kinase, receptor, type 2) • ETV6 (ETS Variant Transcription Factor 6) • NTRK (Neurotrophic receptor tyrosine kinase)
    |
    NTRK positive • NTRK fusion
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    Vitrakvi (larotrectinib) • Rozlytrek (entrectinib)
    4d
    Diagnostic potential of a novel immunohistochemical marker, GFPT2, in differentiating mesothelioma from its morphological mimics. (PubMed, Histopathology)
    This study demonstrates GFPT2's diagnostic utility in MESO, effectively overcoming tumour heterogeneity challenges. It distinguishes malignant mesothelial lesions from benign/borderline ones (RMH/WDPMT), differentiates epithelioid MESO from epithelioid malignances(NSCLC/HGSOC/EHE) and aids in sarcomatoid MESO versus spindle cell tumours (SFT/AF/SS/LMS/MPNST/sarcomatoid carcinoma), though limited for DDLPS. In summary, GFPT2 is a promising novel antibody demonstrating 85.2% sensitivity and 94.7% specificity.
    Journal
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    GFPT2 (Glutamine-Fructose-6-Phosphate Transaminase 2)
    6d
    Expanding the Morphologic and Molecular Spectrum of Spindle Cell Tumors Associated With TERT Fusions. (PubMed, Genes Chromosomes Cancer)
    Thus, our findings suggest that in-frame TERT fusions may drive the pathogenesis of a group of mostly low-grade unclassified sarcomas with fibroblastic/myofibroblastic differentiation. In contrast, TERT fusions may also be detected in other common sarcoma types co-occurring with numerous genomic alterations, likely representing a secondary driver event.
    Journal
    |
    CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • TERT (Telomerase Reverse Transcriptase) • CTNNB1 (Catenin (cadherin-associated protein), beta 1) • CD34 (CD34 molecule)
    |
    CDKN2A deletion
    11d
    Trabectedin-olaparib combination or trabectedin in advanced soft tissue sarcomas after failure of anthracycline-based treatment (TOMAS2): a randomized phase 2 study from the Italian Sarcoma Group. (PubMed, Ann Oncol)
    Although trabectedin-olaparib combination reached the prespecified threshold for statistical significance for PFS (p<0.10), the benefit was marginal in the all-comers STS population. Nonetheless, patients affected by PARP1-expressing STS and uterine leiomyosarcoma derived substantial benefit from the combination, supporting further histology- and biomarker-driven investigation in these settings.
    P2 data • Journal • PARP Biomarker
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    PARP1 (Poly(ADP-Ribose) Polymerase 1)
    |
    Lynparza (olaparib) • Yondelis (trabectedin)
    13d
    LEADER: Lenvatinib and Eribulin in Advanced Soft Tissue Sarcoma (clinicaltrials.gov)
    P1/2, N=30, Completed, National Taiwan University Hospital | Active, not recruiting --> Completed | Trial completion date: Dec 2024 --> Oct 2025
    Trial completion • Trial completion date
    |
    Lenvima (lenvatinib) • Halaven (eribulin mesylate)
    13d
    DUET-1: BOXR1030 T Cells in Subjects With Advanced GPC3-Positive Solid Tumors (clinicaltrials.gov)
    P1/2, N=7, Active, not recruiting, Sotio Biotech Inc. | Trial primary completion date: Oct 2027 --> Oct 2025
    Trial primary completion date • First-in-human
    |
    EGFR (Epidermal growth factor receptor) • ALK (Anaplastic lymphoma kinase) • BRCA (Breast cancer early onset) • GPC3 (Glypican 3)
    |
    EGFR mutation • ALK translocation • BRCA mutation
    |
    cyclophosphamide • BOXR1030
    15d
    N-803 in Patients With Progressive Synovial Sarcoma and Myxoid/Round Cell Liposarcoma Previously Treated With Adoptive Cellular Therapy (clinicaltrials.gov)
    P1, N=8, Not yet recruiting, Northwestern University
    New P1 trial
    |
    Anktiva (nogapendekin alfa inbakicept-pmln)
    16d
    ATRX loss in sarcomas is associated with dysregulated gene and transposable element expression, loss of DNA methylation, and worse survival. (PubMed, medRxiv)
    ATRX status may serve as a potential biomarker for prognosis and therapeutic stratification. Future clinical trials investigating epigenetic therapies could offer novel treatment strategies for ATRX-deficient sarcomas.
    Journal
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    ATRX (ATRX Chromatin Remodeler)
    18d
    Tumor and Immune Dynamics Following Sequential CDK4/6 and PD-1 Inhibition: Results from a Phase 2 Study in Dedifferentiated Liposarcoma. (PubMed, Cancer Res Commun)
    A palbociclib lead-in prior to retifanlimab had a high rate of immune-related toxicities. Correlative analyses identified changes in tumor and immune cells attributable to treatment. A study of concurrent dosing of the combination is ongoing.
    P2 data • Journal • PD(L)-1 Biomarker • IO biomarker
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    LAG3 (Lymphocyte Activating 3) • CD38 (CD38 Molecule) • CD4 (CD4 Molecule) • ICOS (Inducible T Cell Costimulator)
    |
    Ibrance (palbociclib) • Zynyz (retifanlimab-dlwr)
    18d
    Paratesticular liposarcomas: A rare but crucial diagnosis. Case series and review of literature. (PubMed, Cent European J Urol)
    Emerging therapies targeting the MDM2 and CDK4 pathways show promise for advanced or recurrent cases. This report highlights the complexity of diagnosing and managing paratesticular liposarcomas, underlining the importance of multimodal approaches for improved outcomes.
    Review • Journal
    |
    CDK4 (Cyclin-dependent kinase 4)
    20d
    Pathological complete response to pembrolizumab in recurrent retroperitoneal dedifferentiated liposarcoma with high tumor mutational burden: a case report. (PubMed, World J Surg Oncol)
    This is the first reported case of recurrent retroperitoneal DDLPS with high TMB achieving pCR to pembrolizumab. High TMB and high TAM density in the tumor microenvironment may be predictive biomarkers for the response to ICIs in DDLPS.
    Journal • Tumor mutational burden • PD(L)-1 Biomarker • IO biomarker
    |
    PD-L1 (Programmed death ligand 1) • TMB (Tumor Mutational Burden)
    |
    PD-L1 expression • TMB-H • PD-L1 negative
    |
    Keytruda (pembrolizumab) • doxorubicin hydrochloride • pazopanib • Halaven (eribulin mesylate)
    22d
    SOX9, GATA3, and GATA4 Overexpression in Liposarcomas: Insights into the Molecular Biology of Adipocytic Sarcomas. (PubMed, Int J Mol Sci)
    Integration of these molecular markers into diagnostic and prognostic workflows may enhance subtype characterization and inform targeted therapeutic strategies. Further studies in larger cohorts are warranted to validate these biomarkers and explore their mechanistic interplay in liposarcoma pathogenesis.
    Journal
    |
    SOX9 (SRY-Box Transcription Factor 9) • GAPDH (Glyceraldehyde-3-Phosphate Dehydrogenase) • GATA3 (GATA binding protein 3)