^
    17h
    Effects of bone marrow mesenchymal stem cell-conditioned medium on the proliferation and migration of liposarcoma cells. (PubMed, Folia Histochem Cytobiol)
    These findings provide new insights into the impact of BMSC-CM on liposarcoma and suggest its possible involvement in liposarcoma cell growth.
    Journal • IO biomarker
    |
    BCL2 (B-cell CLL/lymphoma 2) • YAP1 (Yes associated protein 1) • MMP2 (Matrix metallopeptidase 2)
    |
    BCL2 expression
    2d
    SAINT:Trabectedin, Ipilimumab and Nivolumab as First Line Treatment for Advanced Soft Tissue Sarcoma (clinicaltrials.gov)
    P1/2, N=45, Recruiting, Sarcoma Oncology Research Center, LLC | Trial completion date: Mar 2024 --> Mar 2025 | Trial primary completion date: Dec 2023 --> Dec 2024
    Trial completion date • Trial primary completion date • IO biomarker • Metastases
    |
    PD-L1 (Programmed death ligand 1) • PD-1 (Programmed cell death 1)
    |
    PD-L1 expression
    |
    Opdivo (nivolumab) • Yervoy (ipilimumab) • Yondelis (trabectedin)
    2d
    LMWH vs Aspirin for VTE Prophylaxis in Orthopaedic Oncology (clinicaltrials.gov)
    P4, N=1434, Enrolling by invitation, Massachusetts General Hospital | Trial completion date: Jul 2024 --> Jul 2028 | Trial primary completion date: Dec 2023 --> Dec 2027
    Trial completion date • Trial primary completion date
    |
    enoxaparin sodium
    2d
    Evaluation of AL3818 in Combination With Nivolumab in Solid Tumors (clinicaltrials.gov)
    P1/2, N=56, Active, not recruiting, Sarcoma Oncology Research Center, LLC | Trial completion date: Mar 2024 --> Mar 2025 | Trial primary completion date: Dec 2023 --> Dec 2024
    Trial completion date • Trial primary completion date • Combination therapy
    |
    Opdivo (nivolumab) • Focus V (anlotinib)
    2d
    Tipifarnib for the Treatment of Advanced Solid Tumors, Lymphoma, or Histiocytic Disorders With HRAS Gene Alterations, a Pediatric MATCH Treatment Trial (clinicaltrials.gov)
    P2, N=5, Active, not recruiting, National Cancer Institute (NCI) | N=49 --> 5 | Trial completion date: Sep 2027 --> Apr 2025 | Trial primary completion date: Sep 2027 --> Mar 2024
    Enrollment change • Trial completion date • Trial primary completion date • Metastases
    |
    Zarnestra (tipifarnib)
    2d
    NTRK-rearranged spindle cell neoplasm: Initial observation of imaging appearance and clinicopathologic correlation. (PubMed, Clin Imaging)
    NTRK-rearranged neoplasms may occur as enhancing masses with linear hypointense signal foci on MRI and FDG avid metastases on PET. Pulmonary metastases were frequent in our study. Initial treatment response is observed in most patients.
    Journal
    |
    NTRK (Neurotrophic receptor tyrosine kinase)
    3d
    Brightline-3: A Study to Find Out Whether Brigimadlin in Combination With Ezabenlimab Helps People With Advanced Soft Tissue Sarcoma (clinicaltrials.gov)
    P3, N=263, Not yet recruiting, Boehringer Ingelheim
    New P3 trial • Combination therapy • Metastases
    |
    gemcitabine • docetaxel • brigimadlin (BI 907828) • ezabenlimab (BI 754091)
    3d
    NeoSarc: Study Evaluating Pembrolizumab +/- Olaparib in TLS Positive Selected Resectable STS Followed by Adjuvant Pembrolizumab (clinicaltrials.gov)
    P2, N=36, Not yet recruiting, Gustave Roussy, Cancer Campus, Grand Paris | Initiation date: Mar 2024 --> Sep 2024
    Trial initiation date
    |
    Keytruda (pembrolizumab) • Lynparza (olaparib)
    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 | Not yet recruiting --> Recruiting | Initiation date: Jul 2023 --> Feb 2024 | Trial primary completion date: Feb 2024 --> Aug 2024
    Enrollment open • Trial initiation date • Trial primary completion date • Metastases
    |
    Focus V (anlotinib) • Kaitanni (cadonilimab)
    3d
    ADVL1622: Cabozantinib-S-Malate in Treating Younger Patients With Recurrent, Refractory, or Newly Diagnosed Sarcomas, Wilms Tumor, or Other Rare Tumors (clinicaltrials.gov)
    P2, N=109, Active, not recruiting, National Cancer Institute (NCI) | Trial completion date: Jul 2023 --> Sep 2024
    Trial completion date
    |
    MET (MET proto-oncogene, receptor tyrosine kinase) • RET (Ret Proto-Oncogene) • AXL (AXL Receptor Tyrosine Kinase)
    |
    MET amplification • MET overexpression • RET mutation • MET mutation • RET rearrangement • AXL overexpression
    |
    Cabometyx (cabozantinib tablet) • Cometriq (cabozantinib capsule)
    4d
    A Trial of Cadonilimab With Adriamycin in Patients With Advanced Soft Tissue Sarcoma (clinicaltrials.gov)
    P2, N=53, Not yet recruiting, Wuhan Union Hospital, China
    New P2 trial • Metastases
    |
    doxorubicin hydrochloride • Kaitanni (cadonilimab)
    4d
    Reclassification of a spindle cell sarcoma after identification of a TFG-ROS1 fusion: A case demonstrating the clinical benefit of next-generation sequencing in sarcoma. (PubMed, Mol Genet Genomic Med)
    We discuss the role of NGS as well as its potential benefit in patients with unresectable, ALK-negative metastatic disease. Considering this case and previous literature, we support the use of NGS for patients requiring systemic treatment.
    Journal • Next-generation sequencing
    |
    ALK (Anaplastic lymphoma kinase) • ROS1 (Proto-Oncogene Tyrosine-Protein Kinase ROS) • CD34 (CD34 molecule) • SOX10 (SRY-Box 10) • USP6 (Ubiquitin Specific Peptidase 6)
    |
    ALK rearrangement • ALK fusion • ROS1 fusion • ROS1 rearrangement • ALK negative • TFG-ROS1 rearrangement
    |
    Xalkori (crizotinib)
    4d
    Lurbinectedin + Doxorubicin In Leiomyosarcoma (clinicaltrials.gov)
    P1/2, N=62, Recruiting, Massachusetts General Hospital | Phase classification: P1b/2 --> P1/2
    Phase classification
    |
    doxorubicin hydrochloride • Zepzelca (lurbinectedin)
    4d
    Radiation Therapy Before Surgery for Localized, Resectable Soft Tissue Sarcoma of the Extremity and Superficial Trunk (clinicaltrials.gov)
    P2, N=120, Active, not recruiting, Mayo Clinic | Recruiting --> Active, not recruiting
    Enrollment closed • Surgery
    5d
    Incisional Negative Pressure Wound Therapy for Preoperatively Irradiated Lower Extremity Soft Tissue Sarcoma Wounds (clinicaltrials.gov)
    P=N/A, N=160, Recruiting, Medical University of South Carolina | N=30 --> 160
    Enrollment change
    5d
    Sequential Neoadjuvant Chemotherapy in Soft Tissue Sarcoma (clinicaltrials.gov)
    P2, N=49, Recruiting, Oslo University Hospital | Trial completion date: Sep 2034 --> Sep 2036 | Trial primary completion date: Sep 2024 --> Sep 2026
    Trial completion date • Trial primary completion date
    |
    doxorubicin hydrochloride • ifosfamide
    7d
    Acquired NF2 mutation confers resistance to TRK inhibition in an ex vivo LMNA::NTRK1-rearranged soft-tissue sarcoma cell model. (PubMed, J Pathol)
    Drug synergy was seen using trametinib and rapamycin in combination with entrectinib.
    Preclinical • Journal
    |
    NTRK1 (Neurotrophic tyrosine kinase, receptor, type 1) • NTRK3 (Neurotrophic tyrosine kinase, receptor, type 3) • NTRK2 (Neurotrophic tyrosine kinase, receptor, type 2) • NF2 (Neurofibromin 2) • LMNA (Lamin A/C) • NTRK (Neurotrophic receptor tyrosine kinase)
    |
    NF2 mutation • NTRK fusion
    |
    Mekinist (trametinib) • Rozlytrek (entrectinib) • sirolimus
    7d
    Feline Facial Spindle Cell Tumors in 29 Cats: Histomorphological and Immunohistochemical Characterization. (PubMed, Animals (Basel))
    Some of these tumors displayed as multiple independent masses on the face or as erosive and ulcerative lesions without obvious mass formation, an atypical presentation and an important highlight for general practitioners, dermatologists, and oncologists. This study also describes periadnexal whorling of neoplastic cells as a novel histomorphologic finding in feline facial PNSTs and emphasizes Sox10 as a useful complementary immunohistochemical marker for the diagnosis of facial PNST in cats, providing valuable insights for veterinary pathologists.
    Journal
    |
    SOX10 (SRY-Box 10) • GFAP (Glial Fibrillary Acidic Protein)
    7d
    Evaluation of the efficacy and safety of immunotherapy in sarcoma: a two-center study. (PubMed, Front Immunol)
    Immunotherapy is an effective treatment modality for sarcoma with manageable safety. Further inclusion of more patients or prospective clinical trials is needed to validate these findings.
    Journal • PD(L)-1 Biomarker • IO biomarker
    |
    PD-L1 (Programmed death ligand 1)
    |
    PD-L1 expression • PD-L1 negative
    8d
    Cabozantinib With Radiation Therapy for the Treatment of Sarcomas of the Extremities (clinicaltrials.gov)
    P1/2, N=6, Active, not recruiting, University of Washington | Recruiting --> Active, not recruiting | N=46 --> 6 | Trial completion date: Jul 2026 --> Jan 2027
    Enrollment closed • Enrollment change • Trial completion date • Combination therapy
    |
    Cabometyx (cabozantinib tablet) • Cometriq (cabozantinib capsule)
    10d
    Abdominopelvic desmoplastic small round cell tumor with metastasis: A case report and literature review. (PubMed, Medicine (Baltimore))
    This case highlights the nonspecific nature of DSRCT symptoms. In clinical practice, it is crucial to meticulously evaluate unexplained intestinal obstruction in young patients, considering DSRCT as a differential diagnosis to avoid delays in diagnosis.
    Review • Journal
    |
    WT1 (WT1 Transcription Factor) • EWSR1 (EWS RNA Binding Protein 1) • VIM (Vimentin) • FLI1 (Fli-1 Proto-Oncogene ETS Transcription Factor) • CDX2 (Caudal Type Homeobox 2)
    11d
    Enzalutamide induces cytotoxicity in desmoplastic small round cell tumor independent of the androgen receptor. (PubMed, Commun Biol)
    Here, we demonstrate that AR is highly expressed in DSRCT relative to other fusion-driven sarcomas and that the AR antagonists enzalutamide and flutamide reduce DSRCT growth. Further, we find that AR antagonists reduce DSRCT growth in cells depleted of AR, establishing an AR-independent mechanism of action. These findings suggest that AR dependence is not the reason for male predominance in DSRCT and that AR-targeted therapies may provide therapeutic benefit primarily through an AR-independent mechanism that requires further elucidation.
    Journal
    |
    AR (Androgen receptor) • WT1 (WT1 Transcription Factor) • EWSR1 (EWS RNA Binding Protein 1)
    |
    AR overexpression
    |
    Xtandi (enzalutamide capsule) • flutamide
    11d
    STRIvE-01: EGFR806 CAR T Cell Immunotherapy for Recurrent/Refractory Solid Tumors in Children and Young Adults (clinicaltrials.gov)
    P1, N=44, Recruiting, Seattle Children's Hospital | Trial completion date: Jun 2038 --> Jun 2040 | Trial primary completion date: Jun 2024 --> Jun 2025
    Trial completion date • Trial primary completion date • CAR T-Cell Therapy
    |
    EGFR (Epidermal growth factor receptor)
    |
    EGFR expression
    |
    EGFR806-specific CAR T-cell therapy • SCRI-CARB7H3(s)x19
    11d
    Comprehensive Transcriptomic Analysis of EWSR1::WT1 Targets Identifies CDK4/6 Inhibitors as an Effective Therapy for Desmoplastic Small Round Cell Tumors. (PubMed, Cancer Res)
    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. 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.
    Journal
    |
    CCND1 (Cyclin D1) • WT1 (WT1 Transcription Factor) • EWSR1 (EWS RNA Binding Protein 1)
    |
    Ibrance (palbociclib)
    11d
    Uncommon Thigh Mass in Neurofibromatosis Type 1: Unveiling Aggressive Epithelioid Sarcoma. (PubMed, Eur J Case Rep Intern Med)
    Patients with neurofibromatosis type 1 or von Recklinghausen's disease have a higher risk than those with other types of neurofibromatosis of developing benign or malignant soft-tissue tumours especially related to the nervous system.Epithelioid sarcoma is an extremely rare subtype of soft-tissue sarcoma and is exceptionally associated with neurofibromatosis type 1.A multidisciplinary approach remains essential in the diagnosis, management, and treatment of soft-tissue sarcomas in patients with neurofibromatosis type 1.
    Journal
    |
    NF1 (Neurofibromin 1)
    12d
    FLASH: Efficacy and Safety of L19TNF in Previously Treated Patients With Advanced Stage or Metastatic Soft-tissue Sarcoma (clinicaltrials.gov)
    P2, N=98, Recruiting, Philogen S.p.A. | Trial primary completion date: Dec 2023 --> Dec 2024
    Trial primary completion date • Metastases
    |
    dacarbazine • Fibromun (onfekafusp alfa)
    12d
    AMPHISARC: HDM201 and Pazopanib in Patients With P53 Wild-type Advanced/Metastatic Soft Tissue Sarcomas (clinicaltrials.gov)
    P1/2, N=58, Recruiting, Centre Leon Berard | Trial completion date: Jul 2025 --> Jul 2026 | Trial primary completion date: Jan 2025 --> Jan 2026
    Trial completion date • Trial primary completion date • Metastases
    |
    MDM2 (E3 ubiquitin protein ligase)
    |
    TP53 wild-type
    |
    Votrient (pazopanib) • siremadlin (HDM201)
    12d
    FIBROSARC: A Study Comparing the Efficacy of L19TNF+Doxorubicin vs Doxorubicin Alone as First-line Therapy in Patients With Advanced or Metastatic Soft Tissue Sarcoma (clinicaltrials.gov)
    P3, N=102, Recruiting, Philogen S.p.A. | Trial completion date: Dec 2024 --> Dec 2025 | Trial primary completion date: Dec 2023 --> Dec 2025
    Trial completion date • Trial primary completion date • Metastases
    |
    doxorubicin hydrochloride • Fibromun (onfekafusp alfa)
    12d
    DOXO75: Dose Finding Study of L19TNF and Doxorubicin in Patients With STS (clinicaltrials.gov)
    P1, N=2, Active, not recruiting, Philogen S.p.A. | Trial completion date: Dec 2023 --> Dec 2024 | Trial primary completion date: Dec 2023 --> Dec 2024
    Trial completion date • Trial primary completion date • Combination therapy • Metastases
    |
    doxorubicin hydrochloride • Fibromun (onfekafusp alfa)
    14d
    Targeting RCC1 to block the human soft-tissue sarcoma by disrupting nucleo-cytoplasmic trafficking of Skp2. (PubMed, Cell Death Dis)
    Notably, recovery of Skp2 expression largely reversed the phenotypes induced by RCC1 knockdown in STS cells. Collectively, this study unveils a novel RCC1-Skp2-p27Kip1 axis in STS oncogenesis, which holds promise for improving prognosis and treatment of this formidable malignancy.
    Journal
    |
    SKP2 (S-phase kinase-associated protein 2)
    15d
    PRONTO: Pre-operative Hypofractionated Proton Therapy (clinicaltrials.gov)
    P=N/A, N=40, Not yet recruiting, Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins | Initiation date: Mar 2024 --> Jun 2024
    Trial initiation date
    16d
    Cutaneous Syncytial Myoepithelioma: An Uncommon and Distinct Variant of Cutaneous Epithelioid Neoplasm. (PubMed, Am J Dermatopathol)
    The histologic features of CSM present a unique set of challenges posing a diagnostic dilemma, as they can bear resemblance to a range of benign and malignant cutaneous neoplasms including ALK-negative epithelioid cell histiocytoma, epithelioid perineurioma, malignant or nevoid melanoma, and epithelioid sarcoma. An accurate diagnosis is crucial for guiding proper clinical management considering that this entity typically demonstrates an excellent prognosis following a complete surgical excision.
    Journal
    |
    ALK (Anaplastic lymphoma kinase) • EWSR1 (EWS RNA Binding Protein 1) • CD34 (CD34 molecule) • TFE3 • SOX10 (SRY-Box 10) • CD68 (CD68 Molecule) • MME (Membrane Metalloendopeptidase) • CD31 (Platelet and endothelial cell adhesion molecule 1) • MLANA (Melan-A) • TP63 (Tumor protein 63) • PECAM1 (Platelet And Endothelial Cell Adhesion Molecule 1) • GFAP (Glial Fibrillary Acidic Protein) • PBX3 (PBX Homeobox 3) • SLC2A1 (Solute Carrier Family 2 Member 1)
    |
    ALK negative
    18d
    Qntrolling the LncRNA HULC-Tregs-PD-1 axis inhibits immune escape in the tumor microenvironment. (PubMed, Heliyon)
    This axis could be modulated to reduce PD-1, IL-10, and TGF-β1 expression and the subsequent immune escape. The inhibition of immune escape in the tumor microenvironment can be achieved by controlling the LncRNA HULC-Treg-PD-1 axis.
    Journal • PD(L)-1 Biomarker • IO biomarker
    |
    IL2RA (Interleukin 2 receptor, alpha) • CD4 (CD4 Molecule) • IL10 (Interleukin 10) • TGFB1 (Transforming Growth Factor Beta 1) • FOXP3 (Forkhead Box P3) • HULC (Hepatocellular Carcinoma Up-Regulated Long Non-Coding RNA)
    |
    PD-1 expression • FOXP3 expression
    19d
    A Study of Tazemetostat in Adult Participants With Soft Tissue Sarcoma (clinicaltrials.gov)
    P2, N=267, Active, not recruiting, Epizyme, Inc. | Trial primary completion date: Jun 2024 --> Feb 2024
    Trial primary completion date
    |
    CD34 (CD34 molecule) • SS18 (SS18 Subunit Of BAF Chromatin Remodeling Complex)
    |
    SMARCA4 mutation
    |
    Tazverik (tazemetostat)
    22d
    A Study of LY2880070 in Participants With Advanced or Metastatic Cancer (clinicaltrials.gov)
    P1/2, N=229, Recruiting, Esperas Pharma Inc. | Phase classification: P1b/2a --> P1/2
    Phase classification • Combination therapy • Metastases
    |
    HER-2 (Human epidermal growth factor receptor 2) • ER (Estrogen receptor) • PGR (Progesterone receptor)
    |
    HER-2 negative • ER expression
    |
    gemcitabine • ESP-01
    23d
    Therapeutic Potential of Bromodomain and Extra-Terminal Domain Inhibitors for Synovial Sarcoma Cells. (PubMed, Cancers (Basel))
    Additionally, knockdown of SS18-SSX, which upregulates BCL2, reduced the sensitivity to ABBV-075. These findings suggest the potential utility of BET inhibitors targeting the SS18-SSX-regulated intrinsic apoptotic pathway as a promising therapeutic strategy for SS.
    Journal • IO biomarker
    |
    BCL2 (B-cell CLL/lymphoma 2) • CDK4 (Cyclin-dependent kinase 4) • BCL2L1 (BCL2-like 1) • CDK6 (Cyclin-dependent kinase 6) • CDKN1A (Cyclin-dependent kinase inhibitor 1A) • SS18 (SS18 Subunit Of BAF Chromatin Remodeling Complex)
    |
    mivebresib (ABBV 075)
    23d
    CEB-01-RLS01-CT: First-in-man Clinical Trial of CEB-01 PLGA Membrane in Recurrent or Locally Advanced Retroperitoneal Soft Tissue Sarcoma (clinicaltrials.gov)
    P1, N=21, Active, not recruiting, CEBIOTEX | Recruiting --> Active, not recruiting | Trial completion date: Mar 2024 --> May 2025 | Trial primary completion date: Dec 2023 --> May 2025
    Enrollment closed • Trial completion date • Trial primary completion date • Surgery • Metastases
    |
    CEB-01
    23d
    Study of PBI-200 in Subjects With NTRK-Fusion-Positive Solid Tumors (clinicaltrials.gov)
    P1, N=29, Terminated, Pyramid Biosciences | Phase classification: P1/2 --> P1
    Phase classification • Metastases
    |
    WT1 (WT1 Transcription Factor) • EWSR1 (EWS RNA Binding Protein 1) • NTRK (Neurotrophic receptor tyrosine kinase)
    |
    WT1 expression • NTRK positive • NTRK fusion • WT1 positive
    |
    PBI-200
    23d
    Targeting refractory/recurrent neuroblastoma and osteosarcoma with anti-CD3×anti-GD2 bispecific antibody armed T cells. (PubMed, J Immunother Cancer)
    This study demonstrated the safety of GD2BATs up to 160×106 cells/kg/infusion. Coupled with evidence of post-treatment endogenous immune responses, our findings support further investigation of GD2BATs in larger phase II clinical trials.
    Clinical Trial,Phase II • Journal
    |
    IFNG (Interferon, gamma) • IL2 (Interleukin 2)
    24d
    Pan-MSI-ACSE: Dostarlimab for Locally Advanced or Metastatic Cancer (Non-colorectal/Non-endometrial) With Tumor dMMR/MSI (clinicaltrials.gov)
    P2, N=120, Not yet recruiting, UNICANCER
    New P2 trial • Metastases
    |
    cisplatin • carboplatin • 5-fluorouracil • doxorubicin hydrochloride • capecitabine • albumin-bound paclitaxel • oxaliplatin • irinotecan • Jemperli (dostarlimab-gxly)
    24d
    UMBRELLA: tislelizUMaB in canceR Patients With molEcuLar residuaL Disease (clinicaltrials.gov)
    P3, N=717, Not yet recruiting, Gustave Roussy, Cancer Campus, Grand Paris
    New P3 trial
    |
    Tevimbra (tislelizumab)
    24d
    NCI-2018-01928: CBL0137 in Treating Patients With Advanced Extremity Melanoma or Sarcoma (clinicaltrials.gov)
    P1, N=7, Terminated, Roswell Park Cancer Institute | N=36 --> 7 | Trial completion date: Aug 2024 --> Jan 2024 | Recruiting --> Terminated | Trial primary completion date: Aug 2024 --> Jan 2024; low accrual
    Enrollment change • Trial completion date • Trial termination • Trial primary completion date • Metastases