^
    18h
    P06 Adenosine deaminase deficient-severe combined immunodeficiency and multicentric dermatofibrosarcoma protuberans: an emerging association. (PubMed, Br J Dermatol)
    Careful dermatological surveillance is warranted in this patient population. Any new or abnormal skin lesions should prompt early referral, histopathological assessment, and tailored surgical management, preferably with Mohs' micrographic surgery or wide local excision.
    Journal
    |
    CD34 (CD34 molecule) • PDGFB (Platelet Derived Growth Factor Subunit B)
    22h
    Hypofractionated vs Conventional Fractionated RT in Soft Tissue Sarcomas (clinicaltrials.gov)
    P2, N=30, Recruiting, University of Wisconsin, Madison | Trial completion date: Nov 2026 --> Nov 2028
    Trial completion date
    1d
    Study of Nivolumab and Ipilimumab in Children and Young Adults With INI1-Negative Cancers (clinicaltrials.gov)
    P2, N=45, Active, not recruiting, Dana-Farber Cancer Institute | Trial primary completion date: Nov 2025 --> Jul 2025
    Trial primary completion date
    |
    SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1)
    |
    Opdivo (nivolumab) • Yervoy (ipilimumab)
    1d
    X-ray Psoralen Activated Cancer Therapy in Head and Neck, Breast, Sarcoma and Melanoma (clinicaltrials.gov)
    P1, N=20, Recruiting, Immunolight, LLC | Trial completion date: Mar 2026 --> Mar 2029 | Trial primary completion date: Sep 2025 --> Sep 2028
    Trial completion date • Trial primary completion date
    |
    methoxsalen (X-PACT)
    1d
    Gemcitabine and Docetaxel With Radiation in Adults With Soft Tissue Sarcoma of the Extremities (clinicaltrials.gov)
    P1, N=9, Terminated, Wake Forest University Health Sciences | N=27 --> 9 | Recruiting --> Terminated; Terminated due to low accruals
    Enrollment change • Trial termination
    |
    gemcitabine • docetaxel
    2d
    Observational Study for Assessing Treatment and Outcome of Patients With Primary Brain Tumours Using cIMPACT-NOW and 2021 WHO Classification (clinicaltrials.gov)
    P=N/A, N=1650, Recruiting, European Organisation for Research and Treatment of Cancer - EORTC | N=1250 --> 1650 | Trial completion date: Dec 2026 --> Jul 2038 | Trial primary completion date: Aug 2026 --> Dec 2027
    Enrollment change • Trial completion date • Trial primary completion date
    3d
    Phase 2 Trial of Zanzalintinib and Pembrolizumab in Select Subtypes of Advanced/Metastatic Soft-tissue Sarcoma (clinicaltrials.gov)
    P2, N=20, Not yet recruiting, M.D. Anderson Cancer Center
    New P2 trial
    |
    Keytruda (pembrolizumab) • zanzalintinib (XL092)
    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
    |
    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
    |
    Vitrakvi (larotrectinib) • Rozlytrek (entrectinib)
    3d
    Efficacy and Safety of Cadonilimab Plus Anlotinib in Advanced STS That Failed the Previous First-line Standard Treatment (clinicaltrials.gov)
    P2, N=27, Recruiting, Second Affiliated Hospital, School of Medicine, Zhejiang University | Trial completion date: Jan 2025 --> Apr 2027 | Trial primary completion date: Aug 2024 --> Apr 2026
    Trial completion date • Trial primary completion date
    |
    Focus V (anlotinib) • Kaitanni (cadonilimab)
    4d
    The first case of GOLGA5-RET fusion-positive malignant spindle cell sarcoma of the head and neck responsive to selpercatinib. (PubMed, Int Cancer Conf J)
    Following confirmation of multiple lung metastases, the patient was treated with doxorubicin monotherapy. Subsequent next-generation sequencing (NGS) revealed a RET fusion, leading to the diagnosis of an RET-rearranged spindle cell neoplasm. This case highlights the importance of genomic testing for certain spindle cell sarcomas and the potential benefit of RET-specific inhibitors against RET-altered sarcomas.
    Journal • IO biomarker
    |
    RET (Ret Proto-Oncogene) • KIT (KIT proto-oncogene, receptor tyrosine kinase) • BCL2 (B-cell CLL/lymphoma 2) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • CDKN2B (Cyclin Dependent Kinase Inhibitor 2B) • CD34 (CD34 molecule) • SOX10 (SRY-Box 10) • NTRK (Neurotrophic receptor tyrosine kinase) • STAT6 (Signal transducer and activator of transcription 6) • GOLGA5 (Golgin A5) • SYP (Synaptophysin)
    |
    RET fusion • RET rearrangement • RET positive
    |
    doxorubicin hydrochloride • Retevmo (selpercatinib)
    4d
    Dermatofibrosarcoma Protuberans of the Chest Wall: A Rare Case. (PubMed, Cureus)
    This case highlights the diagnostic challenges posed by the indolent presentation of DFSP and the importance of early treatment with WLE. Awareness about this rare entity is essential to prevent misdiagnosis and delayed treatment.
    Journal
    |
    CD34 (CD34 molecule) • COL1A1 (Collagen Type I Alpha 1 Chain) • PDGFB (Platelet Derived Growth Factor Subunit B)
    7d
    Thyroid-originated pleomorphic rhabdomyosarcoma with novel TP 53 intron frameshift mutation: a case report and literature review. (PubMed, World J Surg Oncol)
    Under specific circumstances, CNB can provide an effective diagnostic approach for thyroid tumors. Moreover, in this case, we identified a novel TP53 intronic mutation that may drive the development of thyroid PRMS.
    Journal
    |
    BRAF (B-raf proto-oncogene) • TP53 (Tumor protein P53) • NKX2-1 (NK2 Homeobox 1) • MYOD1 (Myogenic Differentiation 1)
    |
    TP53 mutation • BRAF V600E • BRAF V600 • RET mutation • RET rearrangement