^
    1d
    Molecular characterization of adult non-glioblastoma central nervous system (CNS) tumors to identify potential targettable alterations (AIOM 2024)
    4 pts received TT at recurrence, within clinical trials: one with grade 3 meningioma and ALK rearrangement treated with alectinib, one with PTCH1 mutant medulloblastoma treated with vismodegib, and two with high TMB treated with nivolumab/ipilumumab. The incidence of targettable molecular alterations in adult CNS tumor patients was lower than in GBM. Nevertheless, in a few selected cases TT have the potential to increase treatment options at recurrence and improve outcomes.
    Clinical • Tumor mutational burden • BRCA Biomarker • PD(L)-1 Biomarker • IO biomarker
    |
    BRAF (B-raf proto-oncogene) • ALK (Anaplastic lymphoma kinase) • PIK3CA (Phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha) • TMB (Tumor Mutational Burden) • MET (MET proto-oncogene, receptor tyrosine kinase) • BRCA1 (Breast cancer 1, early onset) • BRCA2 (Breast cancer 2, early onset) • RET (Ret Proto-Oncogene) • ROS1 (Proto-Oncogene Tyrosine-Protein Kinase ROS) • FGFR1 (Fibroblast growth factor receptor 1) • PDGFRA (Platelet Derived Growth Factor Receptor Alpha) • NF1 (Neurofibromin 1) • POLE (DNA Polymerase Epsilon) • MDM2 (E3 ubiquitin protein ligase) • PTCH1 (Patched 1) • NF2 (Neurofibromin 2) • BRCA (Breast cancer early onset) • NTRK (Neurotrophic receptor tyrosine kinase)
    |
    BRAF V600E • BRCA2 mutation • BRCA1 mutation • TMB-H • PIK3CA mutation • MET amplification • ALK rearrangement • FGFR1 amplification • POLE mutation • NF1 mutation • MDM2 amplification • RET mutation • PTCH1 mutation • NF2 mutation • ROS1 mutation • BRCA mutation • ALK rearrangement + PIK3CA mutation • PTCH1 rearrangement • NTRK fusion
    |
    FoundationOne® CDx
    |
    Opdivo (nivolumab) • Yervoy (ipilimumab) • Alecensa (alectinib) • Erivedge (vismodegib)
    2d
    Sporadic Breast Angiosarcoma With MYC Amplification on Extrachromosomal Circular DNA Detected Using Nanopore Sequencing in an Adolescent Female. (PubMed, Genes Chromosomes Cancer)
    We also performed methylation classification using nanopore-based methylation data to successfully categorize the tumor as AS. This case report highlights the potential utility of nanopore sequencing in the diagnosis of sarcomas.
    Journal
    |
    MYC (V-myc avian myelocytomatosis viral oncogene homolog)
    |
    MYC amplification
    3d
    Profiling the cancer-prone microenvironment in a zebrafish model for MPNST. (PubMed, Oncogene)
    Finally, we functionally validate a candidate extracellular matrix protein, periostin (POSTN), in human MPNST. This work provides insight into how the microenvironment may regulate MPNST initiation and progression.
    Journal
    |
    POSTN (Periostin)
    3d
    Clinicopathological Analysis of a European Cohort of MYOD1 Mutant Rhabdomyosarcomas in Children and Young Adults. (PubMed, Pediatr Blood Cancer)
    This MYOD1 mutant cohort demonstrates increased MYOD and reduced MYF4 immunostaining, high risk of local failure and poor survival in agreement with other studies. Increased treatment intensity and improved local control should be considered for these patients.
    Journal
    |
    FOXO1 (Forkhead box O1) • MYOD1 (Myogenic Differentiation 1) • PAX3 (Paired Box 3)
    3d
    PTEN pathogenic variants are associated with poor prognosis in patients with advanced soft tissue sarcoma. (PubMed, BJC Rep)
    MutPTEN vs wtPTEN was associated with worse OS in advanced STS. If confirmed, our findings could be helpful for prognostic stratification in clinical practice and for further understanding the molecular mechanisms of STS.
    Journal • Metastases
    |
    TP53 (Tumor protein P53) • PTEN (Phosphatase and tensin homolog) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • RB1 (RB Transcriptional Corepressor 1) • ATRX (ATRX Chromatin Remodeler)
    |
    TP53 mutation • ATRX mutation
    3d
    Talimogene Laherparepvec and Radiation Therapy in Treating Patients With Newly Diagnosed Soft Tissue Sarcoma That Can Be Removed by Surgery (clinicaltrials.gov)
    P2, N=40, Active, not recruiting, National Cancer Institute (NCI) | Trial completion date: Dec 2024 --> Dec 2025 | Trial primary completion date: Dec 2024 --> Dec 2025
    Trial completion date • Trial primary completion date • IO biomarker • Surgery
    |
    PD-L1 (Programmed death ligand 1)
    |
    PD-L1 expression
    |
    Imlygic (talimogene laherparepvec)
    3d
    Advances of SS18-SSX fusion gene in synovial sarcoma: Emerging novel functions and therapeutic potentials. (PubMed, Biochim Biophys Acta Rev Cancer)
    Treatment of synovial sarcoma typically comprises local surgery, radiotherapy and chemotherapy, while novel managements such as immunotherapy, targeted therapies and epigenetic modifiers are explored. This review focuses on the recent studies of SS18-SSX fusion gene, epigenetic landscape, signaling pathways, diagnostic techniques, and relevant therapeutic advances, aiming to inhibit the oncogenic processes and improve outcomes for patients with synovial sarcoma.
    Review • Journal • IO biomarker
    |
    SS18 (SS18 Subunit Of BAF Chromatin Remodeling Complex)
    |
    SS18-SSX fusion
    4d
    Extra-Axial Poorly Differentiated Chordoma Initially Misdiagnosed as Epithelioid Sarcoma. (PubMed, Int J Surg Pathol)
    Given the rarity of this diagnosis, molecular testing was performed which revealed a unique SMARCB1 molecular profile with a single-nucleotide variant in addition to the commonly reported loss of chromosome 22q. This report of an ultra-rare sarcoma in an uncommon anatomic site highlights multiple potential pitfalls in the diagnosis of poorly differentiated chordoma, emphasizes the importance of brachyury immunohistochemistry in rendering a correct interpretation, and underscores an opportunity for further molecular analysis to better define the molecular profile of this entity.
    Journal
    |
    SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1) • GATA3 (GATA binding protein 3)
    4d
    Patterns of expression of VEGFR2, PDGFRs and c-Kit in pediatric patients with high grade non-rhabdomyosarcoma soft tissue sarcoma. (PubMed, Front Oncol)
    Our findings indicated that tyrosine kinase receptors are upregulated in NRSTS and exhibited a distinct expression pattern within various subgroups. High expression of VEGFR2 and PDGFRα significantly correlated with reduced survival and may guide targeted therapy approaches for this poor prognosis group of patients.
    Journal
    |
    KIT (KIT proto-oncogene, receptor tyrosine kinase) • PDGFRA (Platelet Derived Growth Factor Receptor Alpha) • KDR (Kinase insert domain receptor) • PDGFRB (Platelet Derived Growth Factor Receptor Beta)
    |
    KDR overexpression • KDR expression • KIT expression • PDGFRA expression
    4d
    Novel pharmacotherapies for the treatment of liposarcoma: a comprehensive update. (PubMed, Expert Opin Pharmacother)
    The search for novel agents from existing classes (tyrosine kinase inhibitors) with efficacy in liposarcoma also continues. Combination therapies as well as biomarker identification for patient selection of therapies warrant ongoing exploration.
    Review • Journal • IO biomarker
    |
    MDM2 (E3 ubiquitin protein ligase) • CDK4 (Cyclin-dependent kinase 4) • CTAG1B (Cancer/testis antigen 1B)
    4d
    Prognostic factors in clear cell sarcoma: an analysis of soft tissue sarcoma in 43 cases. (PubMed, J Cancer Res Clin Oncol)
    We here present one of the largest clinical cohorts of patients with CCS/GINET. Our data underscores the exceptional risk of metastatic disease even in small tumors. As systemic treatment and radiation showed limited efficacy, complete resection was the most important treatment option.
    Retrospective data • Journal
    |
    EWSR1 (EWS RNA Binding Protein 1) • ATF1 (Activating Transcription Factor 1)
    4d
    Leveraging homologous recombination deficiency for sarcoma : Unravelling homologous recombination repair deficiency and therapeutic opportunities in soft tissue and bone sarcoma. (PubMed, Pathologie (Heidelb))
    We provide a personalized oncological approach to potentially improve the treatment of sarcoma patients. We encourage the evaluation of gene expression signatures to enhance the identification of patients who might benefit from DDR-based therapies.
    Journal • PARP Biomarker
    |
    HRD (Homologous Recombination Deficiency) • RAD51 (RAD51 Homolog A)
    |
    HRD
    5d
    SarcAblate: HIFU Ablation of Soft Tissue Sarcoma (clinicaltrials.gov)
    P=N/A, N=10, Recruiting, Oxford University Hospitals NHS Trust | N=16 --> 10 | Trial completion date: Oct 2025 --> Dec 2026 | Trial primary completion date: Oct 2024 --> Dec 2025
    Enrollment change • Trial completion date • Trial primary completion date
    5d
    Optimizing Genomic Profiling: A Comparative Study of Chromosomal Microarray (CMA) and a 505-Gene NGS Panel for HRD Phenotype Assessment across Diverse Solid Tumor Types Using NxClinical (AMP 2024)
    NGS, with its capacity for comprehensive genomic analysis, excels in detecting diverse genetic alterations, offering superior resolution compared to CMA. This study supports the use of the PGDx elio tissue complete panel as a compelling alternative to CMA, showcasing its accuracy in assessing the HRD phenotype. The observed high concordance rate further enhances its value in the clinical diagnostic setting.
    Next-generation sequencing
    |
    HRD (Homologous Recombination Deficiency)
    |
    HRD • HRD signature
    |
    PGDx elio™ tissue complete assay
    5d
    Radiation Therapy to Treat Musculoskeletal Tumors (clinicaltrials.gov)
    P2, N=202, Active, not recruiting, St. Jude Children's Research Hospital | Trial completion date: Oct 2024 --> Oct 2029
    Trial completion date
    8d
    Continuation or reintroduction of anlotinib plus chemotherapy versus chemotherapy alone in previously treated advanced soft tissue sarcoma: A phase III randomized controlled trial (ChiCTR2400089657)
    P3, N=142, Not yet recruiting, West China Hospital, Sichuan University; West China Hospital, Sichuan University
    New P3 trial • Metastases
    |
    Focus V (anlotinib)
    8d
    A Prospective, Open Phase II Clinical Study Evaluating the Safety and Efficacy of chidamide in Combination with Toripalimab and Anlotinib for the Second-Line Treatment of Advanced or Unresectable Soft Tissue Sarcomas (ChiCTR2400090218)
    P2, N=42, Recruiting, Fudan University Shanghai Cancer Center; Fudan University Shanghai Cancer Center
    New P2 trial • Combination therapy • Metastases
    |
    Focus V (anlotinib) • Loqtorzi (toripalimab-tpzi) • Epidaza (chidamide)
    8d
    A Phase 1 Study Investigating the Safety, Tolerability, Pharmacokinetics, and Preliminary Antitumor Activity of BGB-C354, an Antibody-Drug Conjugate Targeting B7H3, Alone and in Combination With Anti-PD-1 Monoclonal Antibody Tislelizumab in Patients With Advanced Solid Tumors (ChiCTR2400091198)
    P1, N=92, Not yet recruiting, Jilin Cancer Hospital; Jilin Cancer Hospital
    New P1 trial • Combination therapy • Metastases
    |
    EGFR (Epidermal growth factor receptor) • HER-2 (Human epidermal growth factor receptor 2) • BRAF (B-raf proto-oncogene) • ALK (Anaplastic lymphoma kinase) • ROS1 (Proto-Oncogene Tyrosine-Protein Kinase ROS) • CD276 (CD276 Molecule) • NTRK (Neurotrophic receptor tyrosine kinase)
    |
    EGFR mutation • HER-2 negative • EGFR wild-type • CD276 expression
    |
    Tevimbra (tislelizumab-jsgr)
    11d
    Proton Radiation For Meningiomas and Hemangiopericytomas (clinicaltrials.gov)
    P1/2, N=53, Completed, Abramson Cancer Center at Penn Medicine | Active, not recruiting --> Completed
    Trial completion
    11d
    Etiosarc: Environmental Aetiology of Sarcomas From a Multicenter French Population-based Case-control Study Among Adults (clinicaltrials.gov)
    P=N/A, N=2237, Completed, Institut National de la Santé Et de la Recherche Médicale, France | Recruiting --> Completed | N=6462 --> 2237 | Trial completion date: Apr 2022 --> Jul 2024 | Trial primary completion date: Apr 2022 --> Jul 2024
    Trial completion • Enrollment change • Trial completion date • Trial primary completion date
    12d
    Peripheral immune profiling of soft tissue sarcoma: perspectives for disease monitoring. (PubMed, Front Immunol)
    In conclusion, it can be suggested that STS patients have a compromised systemic immunity, and the correlation between immunotypes and survival emphasizes the importance of studying peripheral blood samples in STS. Assessing the peripheral immune response holds promise as a useful method for monitoring and forecasting outcomes in STS.
    Journal
    |
    CD8 (cluster of differentiation 8) • IL10 (Interleukin 10) • GZMB (Granzyme B) • ICOSLG (Inducible T Cell Costimulator Ligand) • ARG1 (Arginase 1) • CD40LG (CD40 ligand) • PRF1 (Perforin 1) • NKG2D (killer cell lectin like receptor K1)
    12d
    Diagnostic challenge: Combination of magnetic resonance imaging and serum soluble Interleukin-2 receptor in soft tissue non-hodgkin lymphoma. (PubMed, J Orthop)
    When both criteria were met, sensitivity and specificity were 72 % and 100 %, respectively. The integrated approach of combining MRI and sIL-2R demonstrated excellent efficacy in predicting the diagnosis of soft tissue NHL, which was initially referred to as soft tissue tumor.
    Journal • MRI
    |
    IL2 (Interleukin 2)
    12d
    BostonGene to Present Research with Leading Cancer Centers at Society for Immunotherapy of Cancer’s Annual Meeting (Businesswire)
    "BostonGene...announced the Company will present six posters at the Society for Immunotherapy of Cancer’s (SITC) 39th Annual Meeting taking place November 6-10, 2024 at the George R. Brown Convention Center in Houston, Texas. The research, conducted in collaboration with leading cancer centers, showcases the impact of BostonGene’s technology in advancing cancer treatment strategies, from deep molecular insights to predictive biomarkers."
    Clinical data • Clinical
    14d
    Study of LVGN3616 and LVGN6051±LVGN7409 in Combination With Nab-Paclitaxel or Bevacizumab and Cyclophosphamide in Metastatic Solid Tumors (clinicaltrials.gov)
    P1, N=352, Active, not recruiting, M.D. Anderson Cancer Center | Recruiting --> Active, not recruiting
    Enrollment closed • Combination therapy • Metastases
    |
    CD40 (CD40 Molecule)
    |
    Avastin (bevacizumab) • albumin-bound paclitaxel • cyclophosphamide • dalnicastobart (LVGN7409) • exlinkibart (LVGN6051) • pradusinstobart (LVGN3616)
    14d
    Proximal and classic epithelioid sarcomas are distinct molecular entities defined by MYC/GATA3 and SOX17/endothelial markers, respectively. (PubMed, Mod Pathol)
    In summary, our study shows that P-ES and C-ES represent distinct molecular entities defined by MYC/GATA3 and SOX17/endothelial molecular traits, respectively. Besides providing insights into the biology of ES, our study pinpoints subtype-specific biomarkers and potential therapeutic vulnerabilities.
    Journal
    |
    EZH2 (Enhancer of zeste 2 polycomb repressive complex 2 subunit) • SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1) • SOX17 (SRY-Box Transcription Factor 17) • CDH2 (Cadherin 2) • GATA3 (GATA binding protein 3)
    |
    EZH2 overexpression
    14d
    PROTraSarc: Prospective Study to Evaluate Patient Reported Outcomes (PRO) During Rechallenge With Trabectedin in Sarcoma Patients (clinicaltrials.gov)
    P=N/A, N=7, Completed, Institut für Klinische Krebsforschung IKF GmbH at Krankenhaus Nordwest | Recruiting --> Completed | N=100 --> 7 | Trial completion date: Dec 2025 --> Jul 2024 | Trial primary completion date: May 2025 --> Jul 2024
    Trial completion • Enrollment change • Trial completion date • Trial primary completion date • Patient reported outcomes
    |
    Yondelis (trabectedin)
    14d
    NEXIS: Neoadjuvant Durvalumab and Tremelimumab Plus Radiation for High Risk Soft-Tissue Sarcoma (clinicaltrials.gov)
    P1/2, N=22, Terminated, University of Maryland, Baltimore | Trial completion date: Jun 2037 --> Jun 2024 | Active, not recruiting --> Terminated | Trial primary completion date: Jun 2032 --> Jun 2024; Limited Accrual
    Trial completion date • Trial termination • Trial primary completion date
    |
    Imfinzi (durvalumab) • Imjudo (tremelimumab)
    17d
    Neurofibroma-like Desmoplastic Melanoma: A Series of Five Cases Exploring the Role of Molecular Testing as a Diagnostic Adjunct and Highlighting the Differential Diagnosis With Diffuse-type Neurofibroma. (PubMed, Am J Surg Pathol)
    Any deviation from the expected clinical or morphologic features of cutaneous diffuse-type neurofibroma should raise suspicion for NFLDM. Although not entirely sensitive or specific, molecular testing can help to support the diagnosis of NFLDM by demonstrating genetic abnormalities associated with melanoma, including a UV-light-induced mutational signature, high tumor mutational burden, and/or chromosomal copy number alterations typical of melanoma.
    Journal • Tumor mutational burden
    |
    TMB (Tumor Mutational Burden)
    |
    TMB-H
    17d
    FAPi Radioligand OpeN-Label, Phase 1 Study to Evaluate Safety, Tolerability and DosImetry of [Lu-177]-PNT6555; A Dose Escalation Study for TReatment of Patients With Select Solid Tumors (FRONTIER) (clinicaltrials.gov)
    P1, N=24, Active, not recruiting, POINT Biopharma, a wholly owned subsidiary of Eli Lilly and Company | N=10 --> 24
    Enrollment change
    17d
    FollowTheDIN: Chemo-immunotherapy in Patients Under 18 Years of Age With Bone and Soft Tissue Sarcomas (clinicaltrials.gov)
    P3, N=40, Recruiting, N.N. Petrov National Medical Research Center of Oncology
    New P3 trial
    |
    carboplatin • docetaxel • temozolomide • doxorubicin hydrochloride • irinotecan • Qarziba (dinutuximab beta)
    17d
    Propranolol Plus Standard Radiation Therapy Before Surgery for the Treatment of Patients With Soft Tissue Sarcoma (clinicaltrials.gov)
    P1, N=12, Not yet recruiting, Roswell Park Cancer Institute
    New P1 trial
    18d
    Palbociclib in Treating Patients With Relapsed or Refractory Rb Positive Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Activating Alterations in Cell Cycle Genes (A Pediatric MATCH Treatment Trial) (clinicaltrials.gov)
    P2, N=23, Completed, National Cancer Institute (NCI) | Active, not recruiting --> Completed
    Trial completion • Metastases
    |
    RB1 (RB Transcriptional Corepressor 1)
    |
    Ibrance (palbociclib)
    18d
    68Ga-DOTATATE PET/CT for the Diagnosis of Soft Tissue Sarcomas (clinicaltrials.gov)
    P=N/A, N=30, Recruiting, Ohio State University Comprehensive Cancer Center | Initiation date: Aug 2024 --> May 2024
    Trial initiation date
    18d
    B7-H3 is widely expressed in soft tissue sarcomas. (PubMed, BMC Cancer)
    These data show high levels of B7-H3 positivity across soft tissue sarcoma subtypes, suggesting its feasibility as a therapeutic target for future sarcoma treatments. Future clinical trials are needed to evaluate whether targeting B7-H3 can provide clinical benefit to help patients with sarcoma.
    Retrospective data • Journal • PD(L)-1 Biomarker • IO biomarker
    |
    PD-L1 (Programmed death ligand 1) • PD-1 (Programmed cell death 1) • CD276 (CD276 Molecule)
    |
    PD-L1 expression • CD276 expression
    19d
    Improving Adolescent and Young Adult Self-Reported Data in ECOG-ACRIN Trials (clinicaltrials.gov)
    P=N/A, N=400, Recruiting, Eastern Cooperative Oncology Group | Trial completion date: Nov 2024 --> May 2027 | Trial primary completion date: Nov 2024 --> May 2027
    Trial completion date • Trial primary completion date
    20d
    Gemcitabine Plus Ascorbate for Sarcoma in Adults (Pilot) (clinicaltrials.gov)
    P1, N=40, Recruiting, University of Iowa | Suspended --> Recruiting
    Enrollment open • Metastases
    |
    gemcitabine
    20d
    Gemcitabine and Docetaxel With Radiation in Adults With Soft Tissue Sarcoma of the Extremities (clinicaltrials.gov)
    P1, N=27, Suspended, Wake Forest University Health Sciences | Trial completion date: Sep 2024 --> Sep 2025 | Trial primary completion date: Sep 2024 --> Sep 2025
    Trial completion date • Trial primary completion date
    |
    gemcitabine • docetaxel
    21d
    Testing the Addition of Cemiplimab to Palbociclib for the Treatment of Advanced Dedifferentiated Liposarcoma (clinicaltrials.gov)
    P2, N=77, Active, not recruiting, Alliance for Clinical Trials in Oncology | Recruiting --> Active, not recruiting
    Enrollment closed
    |
    PD-L1 (Programmed death ligand 1) • RB1 (RB Transcriptional Corepressor 1) • CDK4 (Cyclin-dependent kinase 4)
    |
    PD-L1 expression • RB1 mutation
    |
    Ibrance (palbociclib) • Libtayo (cemiplimab-rwlc)
    21d
    Neoadjuvant Intralesional Injection of Talimogene Laherparepvec (clinicaltrials.gov)
    P1, N=30, Not yet recruiting, John Rieth
    New P1 trial • Metastases
    |
    Imlygic (talimogene laherparepvec)
    21d
    Gemcitabine Plus Ascorbate for Sarcoma in Adults (Pilot) (clinicaltrials.gov)
    P1, N=40, Suspended, University of Iowa | Recruiting --> Suspended
    Trial suspension • Metastases
    |
    gemcitabine
    21d
    A Study of CCT301-59 CAR T Therapy in Adult Subjects With Recurrent or Refractory Solid Tumors (clinicaltrials.gov)
    P1, N=9, Terminated, Shanghai PerHum Therapeutics Co., Ltd. | N=18 --> 9 | Active, not recruiting --> Terminated; Adjustment of study strategy
    Enrollment change • Trial termination
    |
    ROR2 (Receptor Tyrosine Kinase Like Orphan Receptor 2)
    |
    cyclophosphamide • CCT301-59
    22d
    Lymphoid Aggregates in Canine Cutaneous and Subcutaneous Sarcomas: Immunohistochemical and Gene Expression Evidence for Tertiary Lymphoid Structures. (PubMed, Vet Comp Oncol)
    Large LAs were present in 12% of tumours, and LA of any size in 30%. We conclude that large LAs in canine STS are consistent with TLS.
    Journal
    |
    CD20 (Membrane Spanning 4-Domains A1) • CXCL13 (Chemokine (C-X-C motif) ligand 13) • CCL19 (C-C Motif Chemokine Ligand 19) • IRF4 (Interferon regulatory factor 4) • CCL21 (C-C Motif Chemokine Ligand 21) • CXCR5 (C-X-C Motif Chemokine Receptor 5) • LXN (Latexin)