[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"desmoplastic-small-round-cell-tumor\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:desmoplastic-small-round-cell-tumor":28},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,11,0,[8,49,89,114,143,177,205,234,258,292,319],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":14,"acronym":4,"eligibilityCriteria":15,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":18,"enrollmentInfo":19,"targetDuration":4,"studyType":22,"phases":23,"briefSummary":26,"conditions":27,"keywords":4,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":37,"lastUpdatePostDateStruct":38,"startDateStruct":41,"completionDateStruct":43,"leadSponsor":45,"locationsCount":48},"100054232","phase-1-trastuzumab-deruxtecan-ds-8201a-for-the-treatment-of-newly-diagnosed-recurrent-or-refractory-osteosarcoma-wilms-tumor-and-desmoplastic-small-round-cell-tumor-100054232",false,"NCT04616560","Trastuzumab Deruxtecan (DS-8201a) for the Treatment of Newly Diagnosed, Recurrent or Refractory Osteosarcoma, Wilms Tumor, and Desmoplastic Small Round Cell Tumor","A Phase 1\u002F2 Study of DS-8201a (NSC# 807708) in Children, Adolescents, or Young Adults With Recurrent Osteosarcoma, Wilms Tumor, and Desmoplastic Small Round Cell Tumor","Inclusion Criteria:\n\n* Phase 1 (Part A): Patients must be at least 2 years and less than 12 years of age at the time of study enrollment\n* Phase 2: Wilms tumor patients (Part B1): All Wilms tumor patients enrolled must be less than 18 years of age at enrollment\n\n  * Until the completion of the Phase 1 dose confirmation, patients must be at least 12 years of age and less than 18 years of age at the time of study enrollment\n  * Following dose confirmation of DS-8201a in children at least 2 to less than 12 years old in the Phase 1 component, Wilms tumor patients at least 2 to less than 18 years of age will be allowed on the Phase 2 component\n* Phase 2: DSRCT patients (Part B2): Until the completion of the Phase 1 component, patients enrolling on the Phase 2 component of the study must be from at least 12 to 39 years of age at the time of study enrollment\n\n  * Following dose confirmation of DS-8201a in children at least 2 to less than 12 years old in the Phase 1 component, DSRCT patients at least 2 to 39 years of age will be allowed on the Phase 2 component\n* Patients must have had histologic verification of Wilms tumor or desmoplastic small round cell tumor at original diagnosis or relapse\n* Solid tumors: Patients must have measurable disease according to Response Evaluation Criteria in Solid Tumors (RECIST) 1.1. Patients with clinically inactive brain metastases may be included in the study. Patients with treated brain metastases that are no longer symptomatic and who require no treatment with corticosteroids or anticonvulsants may be included in the study if they have recovered from the acute toxic effect of radiotherapy. Lastly, patient must have unresectable lesions or lesions with no intention to surgically remove the lesions in the 6 months following enrollment\n* Wilms tumor: WT patients must have either refractory disease or a very high risk relapse, defined as ANY of the following:\n\n  * Relapse after initial treatment with 4 or more chemotherapy agents (e.g. Regimens vincristine, dactinomycin, doxorubicin, cyclophosphamide, etoposide and radiation \\[M\\], vincristine, dactinomycin and doxorubicin, vincristine, and irinotecan \\[MVI\\], doxorubicin, vincristine, cyclophosphamide, carboplatin, etoposide and radiation \\[UH-1\\], doxorubicin, vincristine, cyclophosphamide, carboplatin, etoposide, vincristine, and irinotecan \\[UH-2\\], vincristine, irinotecan, cyclophosphamide, carboplatin, etoposide and doxorubicin \\[UH-3\\], and etoposide, carboplatin, cyclophosphamide, and doxorubicin \\[HR-1\\])\n  * Relapse with high risk histology (anaplasia, blastemal predominant)\n  * Multiple relapses\n* Desmoplastic small round cell tumor: DSRCT patients with relapsed or refractory disease are eligible\n* Patient's current disease state must be one for which they have received at least standard initial therapy, defined as systemic therapy combined with either radiation or surgery for local control of the primary tumor at diagnosis. Prior therapy after relapse is not required\n* Patients must have a performance status corresponding to Eastern Cooperative Oncology Group (ECOG) scores of 0 or 1. Use Karnofsky for patients older than 16 years of age and Lansky for patients 16 years of age and younger\n* Patients must have recovered from the acute toxic effects of all prior anti-cancer therapy and must meet the following minimum duration from prior anti-cancer directed therapy prior to enrollment. If after the required timeframe, the numerical eligibility criteria are met, e.g., blood count criteria, the patient is considered to have recovered adequately\n\n  * Cytotoxic chemotherapy or other anti-cancer agents known to be myelosuppressive: For agents not listed, the duration of this interval must be discussed with the study chair and the study-assigned Research Coordinator prior to enrollment\n\n    * \\>= 21 days after the last dose of myelosuppressive chemotherapy (42 days if prior nitrosourea)\n  * Anti-cancer agents not known to be myelosuppressive (e.g., not associated with reduced platelet or absolute neutrophil count \\[ANC\\] counts): \\>= 7 days after the last dose of agent\n  * Antibodies: \\>= 4 weeks (28 days) must have elapsed from infusion of last dose of antibody, and toxicity related to prior antibody therapy must be recovered to grade =\\\u003C 1\n  * Corticosteroids\n  * Hematopoietic growth factors: \\>= 14 days after the last dose of a long-acting growth factor (e.g. pegfilgrastim) or 7 days for short-acting growth factor. For agents that have known adverse events occurring beyond 7 days after administration, this period must be extended beyond the time during which adverse events are known to occur\n  * Interleukins, interferons and cytokines (other than hematopoietic growth factors): \\>= 21 days after the completion of interleukins, interferon or cytokines (other than hematopoietic growth factors)\n  * Stem cell Infusions (with or without total body irradiation \\[TBI\\]):\n\n    * Allogeneic (non-autologous) bone marrow or stem cell transplant, or any stem cell infusion including donor lymphocyte infusion (DLI) or boost infusion: \\>= 84 days after infusion and no evidence of graft versus host disease (GVHD)\n    * Autologous stem cell infusion including boost infusion: \\>= 30 days\n  * Cellular therapy: \\>= 30 days after the completion the infusion of any type of cellular therapy (e.g., modified T cells, natural killer \\[NK\\] cells, dendritic cells, etc.)\n  * Radiation therapy (XRT)\u002Fexternal beam irradiation including protons: \\>= 4 weeks (28 days) including palliative radiation therapy to the chest. \\>= 14 days after palliative local XRT to areas other than the chest or for whole brain radiotherapy\n  * Radiopharmaceutical therapy (e.g., radiolabeled antibody, samarium): \\>= 42 days after systemically administered radiopharmaceutical therapy\n  * Patients must not have received prior HER2 therapies including antibody drug conjugates (e.g. TDM-1 or DS-8201a), HER2 directed cellular therapies, HER2 receptor therapy (e.g. trastuzumab, pertuzumab, margetuximab, zanidatamab, zenocutuzumab) or small molecule antagonists of HER2 (e.g lapatinib, tucatinib, or neratinib). Prior exposure to antibody drug conjugates which do not target HER2 as well as prior treatment with topoisomerase 1 inhibitors (e.g. irinotecan, topotecan) are permitted\n* Patients must be at least 14 days from the date of last surgery\n* Peripheral absolute neutrophil count (ANC) \\>= 1000\u002FuL, (for patients with solid tumors without known bone marrow involvement)\n* Platelet count \\>= 100,000\u002FuL (transfusion independent, defined as not receiving platelet transfusions for at least 7 days prior to enrollment) (for patients with solid tumors without known bone marrow involvement)\n* Hemoglobin \\>= 8.0 g\u002FdL at baseline (Red Blood Cell transfusion is not allowed within 1 week prior to enrollment) (for patients with solid tumors without known bone marrow involvement)\n* For patients less than or equal to 17 years old, \"Bedside\" Schwartz formula (2009)\n* Estimated glomerular filtration rate (GFR) (eGFR) ≥ 70 mL\u002Fmin\u002F1.73 m\\^2 (\\> 70mL\u002Fmin\u002F1.73 m\\^2 for patients \\> 17 years old)\n\n  * For patients older than 17 years of age the Cockroft-Gault equation should be utilized to calculate creatinine clearance ≥ 70 ml\u002Fmin:\n  * OR for any age group:\n\n    * A 24 hour urine creatinine clearance ≥ 70 mL\u002Fmin\u002F1.73 m\\^2 (\\> 70mL\u002Fmin for patients ≥ 17 years old) OR\n    * A directly measured GFR ≥ 70 mL\u002Fmin\u002F1.73 m\\^2. GFR must be performed using direct measurement with a nuclear blood sampling method OR direct small molecule clearance method (iothalamate or other molecule per institutional standard)\n* Bilirubin (sum of conjugated + unconjugated or total) =\\\u003C 1.5 x upper limit of normal (ULN) for age. For patients with documented Gilbert's syndrome (unconjugated hyperbilirubinemia) bilirubin must be \\\u003C 3 x ULN for age (patients with solid tumors)\n* Aspartate aminotransferase (AST) =\\\u003C 3 x ULN\n* Serum albumin \\>= 2.5 g\u002FdL (patients with solid tumors)\n* International normalized ratio (INR)\u002Fprothrombin time (PT) =\\\u003C 1.5 x ULN. Exception for patients receiving coumarin-derivative anticoagulants or other similar anticoagulant therapy, who must have INR\u002FPT within the therapeutic range as deemed appropriate by the investigator\n* Shortening fraction of \\>= 27% by echocardiogram, or ejection fraction of \\>= 50% by either an echocardiogram (ECHO) or multigated acquisition (MUGA) scan within 28 days before Step 1 enrollment\n* Corrected QT interval (QTc) prolongation to \\\u003C 480 ms based on average triplicate 12-lead electrocardiogram (ECG)\n* Pulse oximetry \\> 93% on room air\n* Patients with seizure disorder may be enrolled if on anticonvulsants and well controlled as evidenced by no increase in seizure frequency in the prior 7 days\n* Nervous system disorders (Common Terminology Criteria for Adverse Events \\[CTCAE\\] version \\[v\\]5) resulting from prior chemotherapy, surgery, and\u002For radiation must be =\\\u003C grade 2, with the exception of decreased tendon reflex (DTR). Any grade of DTR is eligible\n* HIV-infected patients on effective anti-retroviral therapy with undetectable viral load within 6 months of enrollment are eligible\n* All patients and\u002For their parents or legally authorized representatives must sign a written informed consent. Assent, when appropriate, will be obtained according to institutional guidelines\n\nExclusion Criteria:\n\n* Pregnant, planning to become pregnant, or breast-feeding women will not be entered on this study. Pregnancy tests must be obtained in girls who are post-menarchal. Males or females of reproductive potential may not participate unless they have agreed to use two effective methods of birth control, including a medically accepted barrier or contraceptive method (e.g., male or female condom) for the duration of the study and upon completion of the study and for at least 7 months for females and 4 months for males after the last dose of study drug. Abstinence is an acceptable method of birth control\n\n  * Methods considered as highly effective methods of contraception include:\n\n    * Combined (estrogen and progestogen containing) hormonal contraception associated with inhibition of ovulation:\n\n      * Oral\n      * Intravaginal\n      * Transdermal\n    * Progestogen-only hormonal contraception associated with inhibition of ovulation:\n\n      * Oral\n      * Injectable\n      * Implantable\n    * Intrauterine device (IUD)\n    * Intrauterine hormone-releasing system (IUS)\n    * Bilateral tubal occlusion\n    * Vasectomized partner\n    * Complete sexual abstinence defined as refraining from heterosexual intercourse. Periodic abstinence (calendar, symptothermal, post-ovulation methods) is not an acceptable method of contraception\n* Non-child-bearing potential defined as pre-menopausal females with a documented tubal ligation or hysterectomy; or postmenopausal defined as 12 months of spontaneous amenorrhea (in questionable cases, a blood sample with simultaneous follicle-stimulating hormone \\[FSH\\] \\> 40 mIU\u002FmL and estradiol \\\u003C 40 pg\u002FmL \\[\\\u003C 147 pmol\u002FL\\] is confirmatory). Females on hormone replacement therapy (HRT) and whose menopausal status is in doubt will be required to use one of the contraception methods outlined for women of child-bearing potential if they wish to continue their HRT during the study. Otherwise, they must discontinue HRT to allow confirmation of post-menopausal status prior to study enrollment. For most forms of HRT, at least 2-4 weeks will elapse between the cessation of therapy and the blood draw; this interval depends on the type and dosage of HRT. Following confirmation of their post-menopausal status, they can resume use of HRT during the study without use of a contraceptive method\n* Male patients must not freeze or donate sperm starting at enrollment and throughout the study period, and at least 4 months after the final study drug administration. Preservation of sperm should be considered prior to enrolment in this study\n* Female patients must not donate, or retrieve for their own use, ova from the time of enrollment and throughout the study treatment period, and for at least 7 months after the final study drug administration\n* Patients receiving corticosteroids who have not been on a stable or decreasing dose of corticosteroid for at least 7 days prior to enrollment are not eligible. If used to modify immune adverse events related to prior therapy, \\>= 14 days must have elapsed since last dose of corticosteroid\n* Patients who are currently receiving another investigational drug are not eligible\n* Patients who are currently receiving other anti-cancer agents are not eligible\n* Patients who are receiving cyclosporine, tacrolimus or other agents to prevent graft-versus-host disease post bone marrow transplant are not eligible for this trial\n* Patients who are receiving chloroquine or hydroxychloroquine within 14 days are not eligible for this trial\n* Patients who received a live, attenuated vaccine (messenger ribonucleic acid \\[mRNA\\] and replication deficient adenoviral vaccines are not considered attenuated live vaccines) within 30 days prior to enrollment are not eligible for this trial\n\n  * Note: Participants, if enrolled, should not receive live vaccines during the study and up to 90 days after the last dose of study intervention. It is recommended that patients receive a yearly influenza killed vaccination and additional killed vaccinations based on local or national recommendations. Consider vaccination against viral pathogens that cause pneumonias according to local or national guidelines\n* Patients who have received a prior solid organ transplantation are not eligible\n* Patients with a medical history of myocardial infarction within 180 days before enrollment, symptomatic congestive heart failure (CHF) (New York Heart Association Class II to IV) or troponin levels consistent with myocardial infarction as defined according to the manufacturer 28 days prior to enrollment are not eligible\n* Additionally, patients with a history of any of the following congenital heart disease are not eligible:\n\n  * Single ventricle heart defects (hypoplastic left heart syndrome, unbalanced atrioventricular septal defects, double inlet left ventricle, tricuspid atresia, the presence of superior cavopulmonary anastomosis or Fontan palliation);\n  * Unpalliated defects with significant hemodynamic alterations or palliated lesions with residual hemodynamic alterations (ductal-dependent or shunt-dependent physiology, large unrestrictive ventricular septal defect, transposition of the great arteries, greater than moderate atrioventricular valve insufficiency, moderate or greater aortic valve stenosis, moderate or greater aortic valve insufficiency, large atrial septal defects with significant right ventricular volume overload, large patent ductus arteriosus)\n* Patients who have a pleural effusion, ascites or pericardial effusion that requires drainage, peritoneal shunt, or cell-free and concentrated ascites reinfusion therapy (CART) are not eligible. (Drainage and concentrated ascites reinfusion therapy are not allowed within 2 weeks prior to enrollment)\n* Patients who have spinal cord compression or clinically active central nervous system metastases, defined as untreated and symptomatic, or requiring therapy with corticosteroids or anticonvulsants to control associated symptoms are not eligible\n* Patients with a known history of severe hypersensitivity to DS-8201a, any excipient contained in the DS-8201a drug formulation, or HER2-targeted monoclonal antibodies (trastuzumab, pertuzumab, margetuximab) are not eligible\n* Patients who have an uncontrolled infection or non-healing surgical site are not eligible\n* Patients with a known history of substance abuse or any other clinically significant medical conditions (i.e. psychological conditions) that may, in the opinion of the investigator, interfere with the patient's participation in the clinical study or evaluation of the clinical study results are not eligible\n* Patients who have pulmonary compromise, ex hypoxia, resulting from intercurrent pulmonary illnesses including, but not limited to, any underlying pulmonary disorder (i.e. pulmonary emboli within three months of the study enrollment, severe asthma, severe chronic obstructive pulmonary disease (COPD), restrictive lung disease, pleural effusion etc.), or prior pneumonectomy are not eligible\n* Patients who have a history of (non-infectious) ILD (interstitial lung disease)\u002Fpneumonitis that required steroids, has current ILD\u002Fpneumonitis, or for whom suspected ILD\u002Fpneumonitis cannot be ruled out by imaging at screening are not eligible. Patients who have history of genetic disorders of the lung (i.e. cystic fibrosis are not eligible)\n* Patients who, in the opinion of the investigator, may not be able to comply with the safety monitoring requirements of the study are not eligible\n* Patients with known hepatitis B or C with detectable viral load are not eligible\n* Patients with any autoimmune, connective tissue or inflammatory disorders (e.g., rheumatoid arthritis, Sjogren's, sarcoidosis etc.) where there is documented, or a suspicion of pulmonary involvement at the time of enrollment or genetic diseases involving the lung are not eligible\n* Patients with an active primary immunodeficiency are not eligible","ALL","12 Years","39 Years",{"count":20,"type":21},55,"ESTIMATED","INTERVENTIONAL",[24,25],"PHASE1","PHASE2","This phase I\u002FII trial studies the effects of trastuzumab deruxtecan (DS-8201a) in treating patients with osteosarcoma, Wilms tumor (WT) or desmoplastic small round cell tumor (DSRCT) that is newly diagnosed or has come back after a period of improvement (recurrent) or that has not responded to previous treatment (refractory). Trastuzumab deruxtecan is in a class of medications called antibody-drug conjugates. It is composed of a monoclonal antibody, called trastuzumab, linked to a chemotherapy drug, called deruxtecan. Trastuzumab attaches to HER2 positive tumor cells in a targeted way and delivers deruxtecan to kill them.",[28,29,30,31,32,33,34,35],"Desmoplastic Small Round Cell Tumor","Osteosarcoma","Recurrent Desmoplastic Small Round Cell Tumor","Recurrent Kidney Wilms Tumor","Recurrent Osteosarcoma","Refractory Desmoplastic Small Round Cell Tumor","Refractory Wilms Tumor","Wilms Tumor","RECRUITING","2026-07-10",{"date":39,"type":40},"2026-07-13","ACTUAL",{"date":42,"type":40},"2021-03-08",{"date":44,"type":21},"2027-12-31",{"name":46,"class":47},"National Cancer Institute (NCI)","NIH",28,{"id":50,"slug":51,"hasResults":11,"nctId":52,"briefTitle":53,"officialTitle":54,"acronym":4,"eligibilityCriteria":55,"healthyVolunteers":11,"sex":16,"minAge":56,"maxAge":57,"enrollmentInfo":58,"targetDuration":4,"studyType":22,"phases":60,"briefSummary":61,"conditions":62,"keywords":74,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":78,"lastUpdatePostDateStruct":79,"startDateStruct":81,"completionDateStruct":83,"leadSponsor":85,"locationsCount":88},"100564132","phase-1-alphabeta-t-and-b-cell-depletion-with-zoledronic-acid-for-solid-tumors-100564132","NCT06625190","Alpha\u002FBeta T and B Cell Depletion With Zoledronic Acid for Solid Tumors","Allogeneic Stem Cell Transplantation Utilizing Alpha\u002FBeta T Cell and CD19+ B Cell Depletion With Zoledronic Acid in Combination to Treat Pediatric, Adolescent, and Young Adult Patients With Relapsed\u002FRefractory Solid Tumors","Inclusion Criteria:\n\n* Patients 6 months to ≤ 25 years old\n* Relapsed\u002FRefractory Solid Tumor whom failed or deemed ineligible to receive autologous transplant or if autologous transplant did not offer \\>20% chance of cure with the following diseases:\n\n  1. neuroblastoma (high risk with relapsed or refractory disease),\n  2. relapsed\u002Frefractory rhabdomyosarcoma,\n  3. relapsed\u002Frefractory non-rhabdomyosarcoma soft tissue sarcoma (NRSTS): synovial sarcoma, malignant peripheral nerve sheath tumors (MPNST),\n  4. High risk adult type NRSTS: clear cell sarcoma, alveolar soft part sarcoma,\n  5. Other high-risk extracranial solid tumors: desmoplastic small round cell tumors, chordoma, malignant rhabdoid tumor, epithelioid sarcoma, myoepithelial tumor\n  6. relapsed\u002Frefractory bone tumors: osteosarcoma and Ewing sarcoma\u002FPNET, or\n  7. Wilm's tumor or other high-risk solid tumors with \\\u003C10% expected survival with conventional treatment.\n* Subjects must not have more than one active malignancy at the time of enrollment. (Subjects with a prior or concurrent malignancy whose natural history or treatment does not have the potential to interfere with the safety or efficacy assessment of the investigational regimen \\[as determined by the treating physician and approved by the PI\\] may be included.)\n* Haplo-identical related donor (at least one full haplotype must be matched).\n* Karnofsky or Lansky score ≥60% at the time of enrollment. Karnofsky scores must be used for patients \\>16 years of age and Lansky scores for patients ≤16 years of age\n* Adequate organ function (within 4 weeks of initiation of preparative regimen), defined as:\n\n  1. Pulmonary: FEV1, FVC, and corrected DLCO must all be ≥ 50% of predicted by pulmonary function tests (PFTs). For children who are unable to perform for PFTs due to age, the criteria are: no evidence of dyspnea at rest and no need for supplemental oxygen.\n  2. Renal: Creatinine clearance or radioisotope GFR ≥60 mL\u002Fmin\u002F1.73 m2 or a serum creatinine based on age\u002Fgender\n  3. Cardiac: Ejection fraction of ≥ 40% by echocardiogram or radionuclide scan (MUGA).\n* Written informed consent obtained from the subject and the subject agrees to comply with all the study-related procedures\n* Individuals of childbearing potential (IOCBP) must be using an adequate method of contraception to avoid pregnancy throughout the study and for one year following transplantation to minimize the risk of pregnancy. Prior to study enrollment, individuals of childbearing potential must be advised of the importance of avoiding pregnancy during trial participation and the potential risk factor for an unintentional pregnancy.\n* Subjects with female partners of child-bearing potential must agree to use physician-approved contraceptive methods (e.g., abstinence, condoms, vasectomy) throughout the study and should avoid conceiving children for one year following stem cell transplantation.\n\nExclusion Criteria:\n\n* Patients with documented uncontrolled infection at the time of study entry are not eligible.\n\n  a. Uncontrolled infection is patient without treatment antimicrobials and\u002For demonstrating progression despite antimicrobials\n* Patients with progressive solid tumor disease after relapsed\u002Frefractory treatment.\n* Demonstrated lack of compliance with medical care, as determined by the treating physician.\n* Patients who have received an allogeneic HSCT within 6 months.\n* Patients who do not have an eligible allogeneic donor available.\n* Patients with a life expectancy \\\u003C3 months\n* Patients not meeting inclusion criteria for organ function.\n* Females or males of childbearing potential who are unwilling or unable to use an acceptable method to avoid pregnancy for the entire study period and for at least one year after transplantation.\n* Females who are known to be pregnant or breastfeeding.\n* History of any other disease, metabolic dysfunction, clinical examination finding, or clinical laboratory finding giving reasonable suspicion of a disease or condition that contraindicates the use of protocol therapy or that might affect the interpretation of the results of the study or that puts the subject at high risk for treatment complications, in the opinion of the treating physician.\n* Prisoners or subjects who are involuntarily incarcerated, or subjects who are compulsorily detained for treatment of either a psychiatric or physical illness.","6 Months","25 Years",{"count":59,"type":21},27,[24,25],"Hematopoietic stem cell transplantation can cure patients with blood cancer and other underlying diseases. αβ-T cell and B cell depletion has been introduced to decrease GVHD and PTLD and has demonstrated effectiveness for hematologic malignancies and non-malignant diseases additionally increasing the donor pool as to allow for haploidentical transplant to safely occur.\n\nWhile solid tumors can be highly chemotherapy sensitive, many remain resistant and require multimodalities of treatment. Immunotherapy has been developed to harness the immune system in fighting solid tumors, though not all have targeted effects. Some solid tumors are treated with autologous transplants; however, they do not always demonstrate an improved event free survival or overall survival. There has been evidence of the use of allogeneic stem cell transplants to provide a graft versus tumor effect, though studies remain limited.\n\nBy utilizing αβ-T cell and B cell depletion for stem cell transplants and combining with zoledronic acid, the immune system may potentially be harnessed and enhanced to provide an improved graft versus tumor effect in relapsed\u002Frefractory solid tumors and promote an improved event-free survival and overall survival.\n\nThis study will investigate the safety of treatment with a stem cell graft depleted of αβ-T cell and CD19+ B cells in combination with zoledronic acid in pediatric and young adult patients with select solid tumors, as well as whether this treatment improves survival rates in these patients.",[63,64,65,66,67,68,28,69,70,71,72,29,73,35],"Neuroblastoma","Rhabdomyosarcoma","Synovial Sarcoma","Peripheral Nerve Sheath Tumors","Clear Cell Sarcoma","Alveolar Soft Part Sarcoma","Chordoma","Rhabdoid Tumor","Epithelioid Sarcoma","Myoepithelial Tumor","Ewing Sarcoma",[75,76,77],"pediatric solid tumors","stem cell transplantation","graft manipulation","2026-06-25",{"date":80,"type":40},"2026-06-30",{"date":82,"type":40},"2026-02-11",{"date":84,"type":21},"2030-02",{"name":86,"class":87},"University of Florida","OTHER",1,{"id":90,"slug":91,"hasResults":11,"nctId":92,"briefTitle":93,"officialTitle":94,"acronym":95,"eligibilityCriteria":96,"healthyVolunteers":11,"sex":16,"minAge":97,"maxAge":4,"enrollmentInfo":98,"targetDuration":4,"studyType":22,"phases":100,"briefSummary":101,"conditions":102,"keywords":4,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":103,"lastUpdatePostDateStruct":104,"startDateStruct":106,"completionDateStruct":108,"leadSponsor":110,"locationsCount":113},"100618192","phase-2-clinical-study-in-adult-and-young-adult-patients-with-advanced-desmoplastic-small-round-cell-tumor-dsrct-isg-tulips-100618192","NCT07328425","Clinical Study in Adult and Young Adult Patients With Advanced Desmoplastic Small Round Cell Tumor (DSRCT) ISG-TULIPS","Phase II Study of Lurbinectedin and Irinotecan in Adult and Young Adult Patients With Advanced Desmoplastic Small Round Cell Tumor (DSRCT)","ISG-TULIPS","Inclusion Criteria:\n\n1. Histological centrally confirmed diagnosis of DSRCT with the documented presence of EWSR1-WT1 translocation.\n2. Age ≥ 15 years.\n3. Locally advanced (i.e. radical surgical resection of local disease unfeasible or surgery declined by the patient or surgery deemed to become less demolitive and \u002F or easier after cytoreduction) and\u002For metastatic disease.\n4. Measurable disease by RECIST v1.1.\n5. Clinical or objective disease progression after the last administration of the last standard therapy, or have stopped standard therapy due to intolerability within 6 months from enrollment.\n6. At least one prior chemotherapy based on anthracycline (considering chemotherapy administered for primary tumour) and no more than 3 prior chemotherapy lines.\n7. Eastern Cooperative Oncology Group (ECOG) Performance Status (PS) ≤ 2.\n8. Adequate bone marrow, renal, hepatic, and metabolic function (assessed ≤ 7 days before inclusion in the trial), defined as the following:\n\n   1. platelet count ≥ 100 × 109\u002FL, hemoglobin ≥ 9.0 g\u002FdL, white blood cells ≥ 3.0 × 109\u002FL and absolute neutrophil count (ANC) ≥ 2.0 × 109\u002FL,\n   2. aspartate aminotransferase (AST) and alanine aminotransferase (ALT) ≤ 3.0 × the upper limit of normal (ULN), even in the presence of liver metastases,\n   3. total bilirubin ≤ 1.5 × ULN or direct bilirubin ≤ ULN,\n   4. International Normalized Ratio (INR) \\\u003C 1.5 (except if patient is on oral anticoagulation therapy),\n   5. calculated creatinine clearance (CrCL) ≥ 30 mL\u002Fminute (using Cockcroft-Gault formula),\n   6. creatine phosphokinase (CPK) ≤ 2.5 × ULN,\n   7. albumin ≥ 3.0 g\u002FdL.\n9. Cardiac ejection fraction ≥50% as measured by echocardiogram.\n10. Recovery to grade ≤ 1 or to baseline from any adverse event (AE) derived from previous treatment (excluding alopecia and\u002For cutaneous toxicity and\u002For fatigue grade ≤ 2).\n11. No history of arterial and\u002For venous thromboembolic event within the previous 12 months.\n12. Females of childbearing potential must have a negative pregnancy test (preferable by serum or, if serum test unavailable, urine beta-HCG) within 7 days before treatment start.\n13. Post-menopausal women must be amenorrhoeic for at least 12 months to be considered of non-childbearing potential.\n14. Male and female patients of reproductive potential must agree to employ a highly effective method of birth control (Acceptable methods of contraception are described in Appendix 5) throughout the study and thereafter, at the end of study treatment, and for at least 7 months from the patient's last lurbinectedin administration in female patients of childbearing potential and for at least 4 months in men in fertile age after the last lurbinectedin administration.\n15. The patient or legal representative must be able to read and understand the informed consent form (ICF) and must have been willing to give written informed consent and any locally required authorisation before any study-specific procedures, including screening evaluations, sampling, and analyses.\n\nExclusion Criteria:\n\n1. Prior treatment with lurbinectedin or trabectedin, Ecubectedin (PM 14) or PM54.\n2. Known hypersensitivity to irinotecan or lurbinectedin or any of their components of the drugs products (excipients).\n3. Other primary malignancy with \\\u003C5 years clinically assessed disease free interval, except basal cell skin cancer, cervical carcinoma in situ or other neoplasm judged to entail a low risk of relapse.\n4. History or presence of unstable angina, myocardial infarction, or clinically significant valvular heart disease within 12 months of the study.\n5. Grade III\u002FIV cardiac problems as defined by the New York Heart Association Criteria (i.e. congestive heart failure, myocardial infarction within 12 months of study).\n6. Symptomatic arrhythmia or any uncontrolled arrhythmia requiring ongoing treatment within 12 months of study.\n7. Myopathy or any clinical situation that causes significant and persistent elevation of CPK (\\> 2.5 × ULN in two different determinations performed one week apart).\n8. Severe and\u002For uncontrolled medical disease (i.e. uncontrolled diabetes, chronic renal disease, or active uncontrolled infection).\n9. Known active brain metastasis.\n10. Known chronic liver disease (i.e. chronic active hepatitis and cirrhosis).\n11. Diagnosis of human deficiency virus (HIV), hepatitis C virus (HCV) infection or active hepatitis B (to be excluded during the screening period).\n12. Any past or present chronic inflammatory colon and\u002For liver disease, past intestinal obstruction, pseudo or sub-occlusion or paralysis.\n13. Evident symptomatic pulmonary fibrosis or interstitial pneumonitis, pleural or cardiac effusion rapidly increasing and\u002For necessitating prompt local treatment within seven days.\n14. Any other major illness that, in the Investigator's judgment, will substantially increase the risk associated with the patient's participation in this study.\n15. Known active COVID-19 disease (this includes positive test for SARS-CoV-2 in nasopharyngeal\u002Foropharyngeal swabs or nasal swabs by PCR).\n16. Prior bone marrow and\u002For stem cell transplantation, and allogenic transplant.\n17. Last dose of systemic cytotoxic therapy or investigational therapy within 21 days from enrollment.\n18. Prior treatment with any form of radiation therapy within 14 days from enrollment.\n19. Major surgery within 3 weeks prior to study entry and minor surgery within 1 week prior to study entry.\n20. Use of strong inducers of CYP3A activity within two weeks prior to the first infusion of lurbinectedin (Appendix 6).\n21. Expected limitation of the patient's ability to comply with the treatment or follow-up protocol.\n22. Subjects who have current active hepatic or biliary disease (with exception of patients with asymptomatic gallstones, liver metastasis or stable chronic liver disease per Investigator assessment).\n23. Subjects who have known Gilbert's syndrome.\n24. Patient has received a live or liver attenuated vaccines within 30 days before the first dose of study intervention. Killed vaccines are allowed.","15 Years",{"count":99,"type":21},20,[25],"Patients participating in this study have DSRCT that has spread locally or to other parts of the body and can no longer be surgically removed without causing significant harm.\n\nTreatment will continue until the tumor progresses further, severe side effects occur, or either patient or investigator decision.\n\nIn addition, patients may participate in an optional biological study. The study will analyze the tumor's genes and the molecules related to them. By studying genes and their products, the investigators can better understand the behavior of the tumor and how the body responds to therapies.",[28],"2026-06-09",{"date":105,"type":40},"2026-06-11",{"date":107,"type":40},"2025-11-21",{"date":109,"type":21},"2030-10-01",{"name":111,"class":112},"Italian Sarcoma Group","NETWORK",5,{"id":115,"slug":116,"hasResults":11,"nctId":117,"briefTitle":118,"officialTitle":118,"acronym":4,"eligibilityCriteria":119,"healthyVolunteers":11,"sex":16,"minAge":4,"maxAge":120,"enrollmentInfo":121,"targetDuration":4,"studyType":22,"phases":123,"briefSummary":124,"conditions":125,"keywords":4,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":134,"lastUpdatePostDateStruct":135,"startDateStruct":137,"completionDateStruct":139,"leadSponsor":141,"locationsCount":88},"100431379","phase-1-b7-h3-specific-chimeric-antigen-receptor-autologous-t-cell-therapy-for-pediatric-patients-with-solid-tumors-3car-100431379","NCT04897321","B7-H3-Specific Chimeric Antigen Receptor Autologous T-Cell Therapy for Pediatric Patients With Solid Tumors (3CAR)","Inclusion Criteria:\n\nProcurement and T-cell production eligibility\\*\n\n\\*a previously collected, autologous leukapheresis product can be used for T-cell production\n\n* Age ≤21 years old\n* B7-H3+ solid tumor with measurable disease; B7-H3 expression will be evaluated by standard immunohistochemistry (IHC) using a previously obtained biopsy; a tumor is considered B7-H3 positive with an H-score ≥100\n* Estimated life expectancy of \\>12 weeks\n* Karnofsky or Lansky (age-dependent) performance score ≥50\n* For females of child bearing age:\n* Not pregnant with negative serum pregnancy test within 7 days prior to enrollment\n* Not lactating with intent to breastfeed\n* Meets eligibility criteria to undergo autologous apheresis, or have previously undergone autologous apheresis\n\nExclusion Criteria:\n\n* Known primary immunodeficiency\n* Known HIV positivity\n* Severe intercurrent bacterial, viral or fungal infection (e.g. active hepatitis B or C infection or adenovirus infection)\n* History of hypersensitivity reactions to murine protein-containing products\n* Rapidly progressive disease (in the opinion of the study PIs)\n\nInclusion criteria\n\nTreatment eligibility\n\n* Age ≤21 years old\n* B7-H3+ solid tumor with measurable disease\n* Evidence of relapsed or refractory disease after standard first-line therapy\n* Estimated life expectancy of \\>8 weeks\n* Karnofsky or Lansky (age-dependent) performance score≥50\n* Echocardiogram with a ventricular ejection fraction\n* \\>40%; or shortening fraction ≥25%\n* Adequate renal function defined as creatinine clearance or radioisotope GFR 50 ml\u002Fmin\u002F1.73m2 (GFR 40 ml\u002Fmin\u002F1.73m2 if \\\u003C 2 years of age)\n* Adequate pulmonary function defined as pulse oximetry ≥92% on room air or forced vital capacity (FVC) ≥50% of predicted value\n* Total Bilirubin ≤3 times the upper limit of normal for age, except in subjects with Gilbert's syndrome\n* Alanine aminotransferase (ALT) or aspartate aminotransferase (AST) ≤5 times the upper limit of normal for age\n* Hemoglobin≥ 7g\u002FdL (can be transfused)\n* Platelet count \\>50,000\u002FuL (can be transfused)\n* Absolute neutrophil count (ANC) ≥ 1000\u002FuL\n* Has recovered from all NCI CTAE grade III-IV, non-hematologic acute toxicities from prior therapy\n* For females of child bearing age:\n* Not pregnant with negative serum pregnancy test within 7 days prior to enrollment\n* Not lactating with intent to breastfeed\n* If sexually active, agreement to use birth control until 3 months after T-cell infusion. Male partners should use a condom.\n* Available autologous transduced T-cell product that has met GMP release criteria\n* Agreement to participate in long-term follow-up protocol for patients, who have received genetically modified cell products\n\nExclusion criteria\n\n* Known primary immunodeficiency\n* History of HIV infection\n* Severe, uncontrolled intercurrent bacterial, viral or fungal infection\n* History of hypersensitivity reactions to murine protein-containing products\n* Receiving systemic steroid therapy exceeding the equivalent of 0.5 mg\u002Fkg\u002Fday of methylprednisolone, in the 7 days prior to B7-H3-CAR T-cell infusion\n* Receiving systemic therapy in the 14 days prior to CAR T-cell infusion, which will interfere with the activity of the B7-H3-CAR product (in the opinion of the study PIs).\n* Rapidly progressing disease (in the opinion of the study PIs)","21 Years",{"count":122,"type":21},48,[24],"3CAR is being done to investigate an immunotherapy for patients with solid tumors. It is a Phase I clinical trial evaluating the use of autologous T cells genetically engineered to express B7-H3-CARs for patients ≤ 21 years old, with relapsed\u002Frefractory B7-H3+ solid tumors. This study will evaluate the safety and maximum tolerated dose of B7-H3-CAR T cells.The purpose of this study is to find the maximum (highest) dose of B7-H3-CAR T cells that are safe to give to patients with B7-H3-positive solid tumors.\n\nPrimary objective\n\nTo determine the safety of one intravenous infusion of autologous, B7-H3-CAR T cells in patients (≤ 21 years) with recurrent\u002Frefractory B7-H3+ solid tumors after lymphodepleting chemotherapy\n\nSecondary objective\n\nTo evaluate the antitumor activity of B7-H3-CAR T cells\n\nExploratory objectives\n\n* To evaluate the tumor environment after treatment with B7-H3-CAR T cells\n* To assess the immunophenotype, clonal structure and endogenous repertoire of B7-H3-CAR T cells and unmodified T cells\n* To characterize the cytokine profile in the peripheral blood after treatment with B7-H3-CAR T cells",[126,29,64,63,73,35,127,28,128,70,67,129,130,131,132,133],"Pediatric Solid Tumor","Adrenocortical Cancer","Germ Cell Cancer","Hepatoblastoma","Melanoma","Carcinoma","Malignant Peripheral Nerve Sheath Tumors","Soft Tissue Sarcoma","2026-05-18",{"date":136,"type":40},"2026-05-19",{"date":138,"type":40},"2022-07-06",{"date":140,"type":21},"2028-03-01",{"name":142,"class":87},"St. Jude Children's Research Hospital",{"id":144,"slug":145,"hasResults":11,"nctId":146,"briefTitle":147,"officialTitle":148,"acronym":149,"eligibilityCriteria":150,"healthyVolunteers":11,"sex":16,"minAge":151,"maxAge":4,"enrollmentInfo":152,"targetDuration":4,"studyType":22,"phases":154,"briefSummary":155,"conditions":156,"keywords":159,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":167,"lastUpdatePostDateStruct":168,"startDateStruct":170,"completionDateStruct":172,"leadSponsor":174,"locationsCount":176},"100509838","phase-1-lurbinectedin-in-fet-fused-tumors-100509838","NCT05918640","Lurbinectedin in FET-Fused Tumors","Lurbinectedin in FET-Fusion Tumors (LIFFT)","LiFFT","Inclusion Criteria:\n\n1. Age ≥ 10 years.\n2. Phase 1: Histological confirmed diagnosis of recurrent or relapsed solid tumor failing primary therapy. Patients must have a known FET fusion (fusion that contains EWSR1, FUS, or TAF15) as documented by next generation sequencing, polymerase chain reaction (PCR) or Fluorescence in situ hybridization (FISH). Patients with a histological diagnosis of Ewing sarcoma with EWS-FLI1 are eligible for dose escalation but not for the exploratory cohort. Please note patients with Ewing sarcoma and alternative FET-ETS fusions (including but not limited to EWS-ERG, EWS-ETV1, EWS-ETV4, EWS-FEV, FUS-ERG, FUS-FEV) are eligible for the exploratory cohort.\n3. Phase 2: Histologically confirmed diagnosis of recurrent or relapsed Ewing sarcoma failing primary therapy with confirmation of EWS-FLI1 fusion and breakpoint by Next generation sequencing or PCR or EWSR1 rearrangement confirmed by FISH and available tissue for central confirmation of EWS-FLI1 fusion and breakpoint.\n4. Eastern Cooperative Oncology Group (ECOG) Performance Status of 0 or 1 (age \\>16 years) or Lansky of at least 70 (age \\\u003C16 years).\n5. Disease status (baseline imaging must be performed within 28 days of Day 1 of study treatment):\n\n   1. Phase 1: At least one site of measurable disease on CT or MRI as defined by RECIST 1.1 OR evaluable disease with at least one site of disease that has not been previously radiated\n   2. Phase 2: At least one site of measurable disease on CT or MRI as defined by RECIST 1.1\n6. Meets organ function requirements as outlined below:\n\n   1. Liver:\n\n      Alanine aminotransferase (ALT) ≤ 2.5X upper limit of normal. For the purposes of this study the upper limit of normal for ALT is 45 U\u002FL. Aspartate aminotransferase (AST) ≤ 2.5X upper limit of normal. For the purposes of this study the upper limit of normal for AST is 50 U\u002FL. Total bilirubin ≤ 1.5X institutional upper limit of normal with the exception of patients with Gilbert's syndrome who must have bilirubin \\\u003C3X institutional upper limit of normal.\n   2. Renal:\n\n      Creatinine Calculated creatinine clearance (by the Schwartz equation for patients \\\u003C18 years of age and Cockroft-Gault formula (Appendix B) for patients ≥18 years of age) or radionuclide glomerular filtration rate (GFR) ≥ 50 mL\u002Fmin \u002Fm2 or a serum creatinine less than or equal to the age\u002Fgender valued below:\n\n      Age Maximum Serum Creatinine (mg\u002FdL) Male Female 10 to \\\u003C 13 years 1.2 1.2 13 to \\\u003C 16 years 1.5 1.4\n\n      ≥ 16 years 1.7 1.4\n   3. Bone marrow:\n\n      Absolute Neutrophil Count (ANC) ≥ 1,000\u002FµL (\\>one week since last dose of short acting medications (e.g. filgrastim) and \\> two weeks since last dose of long acting medications (e.g. peg-filgrastim)) Platelet Count (PLTs) ≥ 100,000\u002F µL (\\>two weeks since last dose of thrombopoietin receptor agonist such as romiplostim and without platelet transfusion within previous 7 days of screening laboratories) Patients with a history of bone marrow involvement are required to have bilateral bone marrow aspirates and biopsies at baseline. Subjects with bone marrow disease are eligible as long as they meet the hematologic requirements above and are not known to be refractory to red cell or platelet transfusions.\n   4. Muscle: Creatine phosphokinase (CPK) CTCAE 5 Grade ≤ 1,\n   5. Cardiac:\n\n   Creatine phosphokinase CTCAE 5 Grade ≤ l, Left ventricular ejection fraction (LVEF) or shortening fraction (SF) per institutional norm LVEF ≥50% OR SF ≥28%.\n7. Written, voluntary informed consent\n8. Fertile males and females of childbearing potential must agree to use an effective method of birth control from screening, through 7 months after last study drug administration for females and 4 months for males. Women of childbearing potential must have a negative pregnancy test during screening procedures. Effective methods of birth control include: double barrier method (condom, diaphragm), abstinence, an intrauterine device (IUD), levonorgestrol implants, medroxyprogesterone acetate injections, or oral contraception. For those subjects whose preferred and usual lifestyle employs abstinence, refraining from heterosexual intercourse must be practiced during the entire active phase of the trial.\n9. Patients ≥18 years must be willing to undergo tumor biopsy at study entry. Patients with Ewing sarcoma or DSRCT must be willing to undergo biopsy post-treatment. If biopsy is contraindicated, enrollment must be approved by study PI and archival tissue must be available.\n10. Time elapsed from previous therapy:\n\n    1. Must be ≥ 3 weeks for systemic myelosuppressive therapy\n    2. ≥ 2 weeks for local radiation therapy (small field), ≥ 150 days after thyrotropin binding inhibition (TBI), craniospinal external beam radiotherapy (XRT) or radiation to ≥50% of the pelvis\n    3. ≥ 2 weeks for major surgery\n    4. ≥ 2 weeks for monoclonal antibodies and oral kinase inhibitors.\n    5. ≥ 6 weeks for autologous stem cell transplant. 6 months for allogeneic stem cell transplant.\n    6. ≥ 6 weeks for any type of cellular therapy\n11. Patients must be recovered to baseline or Grade ≤1from the acute adverse effects of prior treatments, with the exception of alopecia and decreased deep tendon reflexes.\n\nExclusion Criteria:\n\n1. Prior therapy with trabectedin or lurbinectedin.\n2. Subjects with known brain metastases.\n3. Subjects with a known bleeding diathesis.\n4. Subjects who are pregnant or breastfeeding.\n5. Concurrent therapy:\n\n   1. Patients who are currently receiving an investigational drug or another anticancer agent\n   2. Patients receiving over the counter or herbal supplement with significant potential hepatotoxicity in the opinion of the investigator.\n   3. Patients receiving a medically necessary strong or moderate CYP3A4 inhibitor or inducer within 14 days prior to the first dose of study drug.\n6. Clinically significant, unrelated illness or uncontrolled infection which would, in the opinion of the treating physician, compromise the patient's ability to tolerate the investigational agents or be likely to interfere with the study procedures or results.\n7. Subjects who, in the opinion of the investigator, may not be able to comply with the safety monitoring requirements of the study, or in whom compliance is likely to be suboptimal, should be excluded.\n8. Patients with known active viral hepatitis (i.e. Hepatitis A, B, or C)\n9. Patients with Desmoplastic small round cell tumor (DSRCT) will be excluded from enrollment until at least 3 non-DSRCT patients have been enrolled without dose limiting toxicity.","10 Years",{"count":153,"type":21},63,[24,25],"The purpose of this study is to find out if a drug called lurbinectedin (the \"study drug\") is safe and effective at treating people with recurrent or relapsed solid tumors, including Ewing sarcoma.",[73,28,157,158],"Pediatric Cancer","Undifferentiated Sarcoma",[160,161,162,163,164,165,166],"Ewing Sarcoma-Friend Leukemia Integration 1 Transcription factor (ESW-FLI1)","Ewing Sarcoma Breakpoint Region 1-Friend Leukemia Integration 1 Transcription factor (EWSR1-FLI1)","Ewing Sarcoma Erythroblast Transformation Specific Related Gene (EWS-ERG)","Ewing Sarcoma Breakpoint Region 1 (EWRS1)","TATA-Box-Binding Protein Associated Factor 15 (TAF15)","Fused Tumors (FET)","Ewing Sarcoma-Wilms' Tumor Gene 1 (EWS-WT1)","2026-03-26",{"date":169,"type":40},"2026-03-31",{"date":171,"type":40},"2023-07-27",{"date":173,"type":21},"2028-07-30",{"name":175,"class":87},"Children's Hospital of Philadelphia",6,{"id":178,"slug":179,"hasResults":11,"nctId":180,"briefTitle":181,"officialTitle":181,"acronym":4,"eligibilityCriteria":182,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":183,"enrollmentInfo":184,"targetDuration":4,"studyType":22,"phases":185,"briefSummary":186,"conditions":187,"keywords":191,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":195,"lastUpdatePostDateStruct":196,"startDateStruct":198,"completionDateStruct":200,"leadSponsor":202,"locationsCount":204},"100570611","phase-1-phase-12-trial-to-evaluate-the-safety-and-efficacy-of-peel-224-in-combination-with-vincristine-and-temozolomide-in-adolescents-and-young-adults-with-relapsed-or-refractory-sarcomas-100570611","NCT06709495","Phase 1\u002F2 Trial to Evaluate the Safety and Efficacy of PEEL-224 in Combination With Vincristine and Temozolomide in Adolescents and Young Adults With Relapsed or Refractory Sarcomas","Inclusion Criteria:\n\n-Patients in all cohorts must have relapsed or refractory disease after standard therapy.\n\nInclusion Criteria Phase 1 (only) diagnosis requirements:\n\n-Patients must have:\n\n* Evaluable or measurable disease; and\n* Histologic diagnosis of sarcoma\n\nInclusion Criteria Phase 2 (only) diagnosis requirements\n\n* EWS cohort: Patients must have:\n\n  * RECIST measurable disease at study entry;\n  * Histologic diagnosis consistent with Ewing sarcoma; and\n  * Molecular evidence of a FET-ETS family translocation including but not limited to any of the following:\n\n    * EWSR1::FLI1, EWSR1::ERG, EWSR1::ETV1, EWSR1::ETV4, EWSR1::FEV, FUS::FLI1, FUS::ERG\n* DSRCT cohort: Patients must have:\n\n  * RECIST measurable disease at study entry;\n  * Histologic diagnosis consistent with DSRCT; and\n  * Molecular evidence of an EWSR1::WT1 fusion\n* Other sarcoma cohort: Patients must have:\n\n  * RECIST evaluable or measurable disease; and\n  * Histologic diagnosis of sarcoma. Patients with EWS or DSRCT with evaluable but not measurable disease may participate in this cohort.\n  * Slots in this cohort will include three dedicated slots for patients with rhabdomyosarcoma, three dedicated slots for patients with osteosarcoma and three dedicated slots for patients with other translocation-associated round cell sarcomas.\n* Age: ≥ 12 years and ≤ 49 years.\n* Weight: Patients must be ≥ 40 kg.\n* Performance Status: Karnofsky ≥ 50% for patients \\>16 year of age and Lansky ≥ 50% for patients ≤ 16 years of age. (see Appendix A for definitions of Lansky and Karnofsky Performance Status).\n* Participants must meet the following organ and marrow function as defined below: Adequate Bone Marrow Function:\n\n  * Hematologic Requirements for Subjects without Bone Marrow Involvement by\n\nDisease:\n\n* Absolute neutrophil count (ANC) ≥ 1,000\u002FuL\n* Platelet count ≥100,000\u002FuL (transfusion independent, defined as not receiving platelet transfusions for at least 7 days prior to enrollment)\n\n  --Hematologic Requirements for Subjects with Bone Marrow Involvement by Disease:\n* ANC ≥750 \u002FuL\n* Platelets ≥50,000 \u002FuL (may receive platelet transfusions) Not known to be refractory to red cell and\u002For platelet transfusions.\n\n  --Adequate Renal Function: Creatinine clearance or radioisotope GFR ≥70ml\u002Fmin\u002F1.73 m2 or A serum creatinine based on age\u002Fsex as follows:\n* Age: 12 to \\\u003C 13 years, Maximum Serum Creatinine (mg\u002FdL): Male 1.2, Female 1.2\n* Age 13 to \\\u003C 16 years, Maximum Serum Creatinine (mg\u002FdL): Male 1.5, Female 1.4\n\n  ---≥ 16 years, Maximum Serum Creatinine (mg\u002FdL): Male 1.7, Female 1.4\n\n  --Adequate Liver Function:\n* Bilirubin (sum of conjugated + unconjugated) ≤ 1.5 x upper limit of normal (ULN) for age\n* SGPT (ALT) ≤110 U\u002FL. For the purpose of this study, the ULN for SGPT is 45 U\u002FL.\n\n  * Adequate Cardiac Function: QTc \\\u003C 480 msec\n\n    -Patients must have fully recovered from the acute toxic effects of all prior anti-cancer therapy except organ function as noted above. Patients must meet the following minimum washout periods prior to enrollment:\n  * Myelosuppressive chemotherapy: At least 14 days after the last dose of myelosuppressive chemotherapy\n  * Radiotherapy:\n* At least 14 days after local XRT (small port, including cranial radiation);\n* At least 90 days must have elapsed after prior TBI, craniospinal XRT or if \\>50% radiation of pelvis;\n* At least 42 days must have elapsed if other substantial BM radiation.\n\n  * Small molecule biologic therapy: At least 7 days following the last dose of a biologic agent.\n  * Monoclonal antibody: At least 21 days must have elapsed after the last dose of antibody.\n  * Myeloid and platelet growth factors: At least 14 days following the last dose of long-acting myeloid growth factor (e.g. Neulasta) or 7 days following short-acting myeloid or platelet growth factor.\n  * Autologous hematopoietic stem cell transplant and stem cell boost: Patients must be at least 60 days from day 0 of an autologous stem cell transplant or stem cell boost.\n  * Cellular Therapies (e.g., CART, NK-cell based therapy): The patient must be and at least 42 days from cellular therapy administration.\n  * Major Surgery: At least 2 weeks from prior major surgical procedure. Note: Biopsy, CNS shunt placement\u002Frevision, and central line placement\u002Fremoval are not considered major.\n  * Irinotecan, liposomal irinotecan, and\u002For temozolomide: Patients may have received prior irinotecan, liposomal irinotecan, and\u002For temozolomide. NOTE: Patients who have had progressive disease while receiving irinotecan and temozolomide in combination will be excluded from the Phase 2 EWS and DSRCT cohorts only.\n\n    -For patients with metastatic disease to the CNS enrolling to the phase 1 portion of the trial or the \"other sarcoma\" cohort, any baseline neurologic deficits (including seizure) must be stable for at least one week prior to study enrollment. Patients with CNS metastatic disease receiving corticosteroids must be on a stable or decreasing dose at time of study entry.\n  * Patients with CNS metastatic disease will not be eligible for the phase 2 EWS and DSRCT cohorts.\n\n    * Participants with a prior or concurrent malignancy whose natural history or treatment does not have the potential to interfere with the safety or efficacy assessment of the investigational regimen are eligible for this trial.\n    * The effects of PEEL-224 in combination with temozolomide and vincristine on the developing human fetus are unknown. For this reason, women of child-bearing potential and men must agree to use adequate contraception (hormonal or barrier method of birth control; abstinence) prior to study entry and for the duration of study participation. Should a woman become pregnant or suspect she is pregnant while she or her partner is participating in this study, she should inform her treating physician immediately. Men treated or enrolled on this protocol must also agree to use adequate contraception prior to the study, for the duration of study participation, and 4 months after completion of PEEL-224 administration.\n    * Ability to understand and\u002For the willingness of the patient (or parent or legally authorized representative, if minor) to provide informed consent, using an institutionally approved informed consent procedure.\n    * Any participant must obtain prior approval from insurance to reimburse oral temozolomide for the duration of the study or agree to self-pay for oral temozolomide.\n\nExclusion Criteria:\n\n* Patients who have received prior treatment with PEEL-224.\n* Patients who have had progressive disease while receiving irinotecan and temozolomide in combination will be excluded from the Phase 2 EWS and DSRCT cohorts only.\n* Participants who are receiving any other anti-cancer agents for this condition.\n* Patients receiving strong P450 CYP1A2 and CYP3A4 inhibitors and\u002For inducers with 14 days of the first planned dose of PEEL-224. NOTE: levofloxacin is permitted and preferred over ciprofloxacin for patients needing a fluoroquinolone.\n* Patients who have received a solid organ or allogeneic stem cell transplant\n* Pregnant participants, given that the effects of PEEL-224 on the developing human fetus are unknown.\n* Breastfeeding mothers, because there is an unknown risk for adverse events in nursing infants secondary to treatment of the mother with PEEL-224.\n* Patients with a history of allergic reactions attributed to PEGylated drugs, camptothecins, temozolomide or vincristine.\n* Patients with uncontrolled intercurrent illness including, but not limited to, ongoing or active infection, symptomatic congestive heart failure, unstable angina pectoris, cardiac arrhythmia, or psychiatric illness\u002Fsocial situations that would limit compliance with study requirements.","49 Years",{"count":153,"type":21},[24,25],"This research is being done to test a new drug called PEEL-224 in combination with two commercially available drugs, Vincristine and Temozolomide, and to determine how effective this combination of drugs is at treating Ewing Sarcoma (EWS) and Desmoplastic Small Round Cell Tumor (DSRCT), as well as multiple other kinds of sarcomas.\n\nThe names of the study drugs and biological agents involved in this study are:\n\n* PEEL-224 (a type of Topoisomerase 1 inhibitor)\n* Vincristine (A type of vinca alkaloid)\n* Temozolomide (A type of alkylating agent)\n* Pegfilgrastim or Filgrastim (types of Myeloid growth factors)",[188,189,28,190,29,64],"Sarcoma","Sarcoma, Ewing","Refractory Sarcoma",[188,189,28,190,192,193,194],"Relapsed Sarcoma","osteosarcoma","rhabdomyosarcoma","2026-02-17",{"date":197,"type":40},"2026-02-19",{"date":199,"type":40},"2025-01-27",{"date":201,"type":21},"2029-09-01",{"name":203,"class":87},"David S Shulman, MD",3,{"id":206,"slug":207,"hasResults":11,"nctId":208,"briefTitle":209,"officialTitle":209,"acronym":210,"eligibilityCriteria":211,"healthyVolunteers":11,"sex":16,"minAge":212,"maxAge":4,"enrollmentInfo":213,"targetDuration":4,"studyType":22,"phases":215,"briefSummary":217,"conditions":218,"keywords":224,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":225,"lastUpdatePostDateStruct":226,"startDateStruct":228,"completionDateStruct":230,"leadSponsor":232,"locationsCount":99},"100360033","multimodal-immune-characterization-of-rare-soft-tissue-sarcoma---miras-project-from-sarra-sarcome-rare-project-of-the-french-sarcoma-group-100360033","NCT03967834","Multimodal Immune Characterization of RAre Soft Tissue Sarcoma - MIRAS Project From SARRA (SARcome RAre) Project of the French Sarcoma Group","MIRAS","Inclusion Criteria:\n\n1. Age ≥18 years at the time of study entry.\n2. Diagnosis of one of the following rare sarcoma subtype, confirmed by RRePS network:\n\n   * Clear Cell Sarcoma (CCS)\n   * Epithelioid Sarcoma (ES)\n   * Perivascular Epithelioid Cell neoplasm (PEComa)\n   * Desmoplastic Small Round Cell Tumours (DSRCT)\n   * Malignant Solitary Fibrous Tumours (mSFT)\n   * Alveolar Soft Part Sarcoma (ASPS)\n   * Epithelioid Hemangioendothelioma (EH)\n   * Low-Grade Fibromyxoid Sarcoma (LGFS)\n   * Sclerosing Epithelioid Fibrosarcoma (SEF).\n3. Localized\u002Flocally advanced or metastatic disease.\n4. In case of localized disease, treatment must not have been yet initiated before inclusion (except surgical excision).\n5. In case of metastatic disease, project of new line of systemic treatment must have been decided before inclusion.\n6. Patient followed in the center within a standard of care procedure or clinical trial.\n7. Archived tumor specimen at initial diagnosis available (before treatment initiation).\n8. Evaluable disease (measurable as per RECIST 1.1) or not.\n9. ECOG Performance status 0-3.\n10. Patient able to participate and willing to give informed consent prior to performance of any study-related procedures.\n11. Patient affiliated to a Social Health Insurance in France.\n\nExclusion Criteria:\n\n1. Diagnosis of all other histotypes of soft tissue sarcoma.\n2. Any condition contraindicated with procedures required by the protocol.\n3. Known history of positive test for hepatitis B virus or hepatitis C virus or human immunodeficiency virus (HIV) or known acquired immunodeficiency syndrome (AIDS).\n4. Any psychological, familial, geographic or social situation, according to the judgment of investigator, potentially preventing the provision of informed consent or compliance to study procedure.\n5. Pregnant or breast-feeding woman.\n6. Patient who has forfeited his\u002Fher freedom by administrative or legal award or who is under guardianship.","18 Years",{"count":214,"type":21},400,[216],"NA","This trial is a translational, open-label, multi-sites, prospective and retrospective cohort study of 500 patients aimed at clinical and biological characterization of sarcoma of rare subtype.\n\n400 patients will be included in this prospective cohort study; they will be identified in the investigating centers in the context of either routine care or a clinical study protocol.\n\nRetrospective cases of patients (100 cases in total) will be identified in all centers through the GSF\u002FGETO clinical databases already setted up (including the clinical base Conticabase).",[133,67,71,219,28,220,68,221,222,223],"Perivascular Epithelioid Cell Neoplasms","Malignant Solitary Fibrous Tumors","Epithelioid Hemangioendothelioma","Low Grade Fibromyxoid Sarcoma","Sclerosing Epithelioid Fibrosarcoma",[133,67,71,219,28,220,68,221,222,223],"2026-02-09",{"date":227,"type":40},"2026-02-10",{"date":229,"type":40},"2021-04-26",{"date":231,"type":21},"2031-04",{"name":233,"class":87},"Institut Claudius Regaud",{"id":235,"slug":236,"hasResults":11,"nctId":237,"briefTitle":238,"officialTitle":239,"acronym":4,"eligibilityCriteria":240,"healthyVolunteers":11,"sex":16,"minAge":4,"maxAge":4,"enrollmentInfo":241,"targetDuration":151,"studyType":243,"phases":4,"briefSummary":244,"conditions":245,"keywords":246,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":249,"lastUpdatePostDateStruct":250,"startDateStruct":252,"completionDateStruct":254,"leadSponsor":256,"locationsCount":88},"100415495","registry-to-collect-health-information-about-desmoplastic-small-round-cell-tumor-100415495","NCT04690374","Registry to Collect Health Information About Desmoplastic Small Round Cell Tumor","Registry for Patients With Desmoplastic Small Round Cell Tumor","Inclusion Criteria:\n\n* Participants must have a diagnosis of desmoplastic small round cell tumor\n* Participants may be of any age as long as the appropriate consent and assent may be obtained\n* Willing to provide historical and longitudinal clinical data\n\nExclusion Criteria:\n\n* Participant unwilling to provide consent or share historical and longitudinal clinical data",{"count":242,"type":21},250,"OBSERVATIONAL","This study is a patient registry of people with Desmoplastic Small Round Cell Tumor (DSRCT). A patient registry is a collection of health information about a group of people, and it is usually focused on a specific diagnosis or disease.\n\nThe purpose of this registry is to create a database- a collection of information-or better understanding DSRCT. Researchers will use the information from this database to learn more about DSRCT and for current and future research on DSRCT.",[28],[247,248],"Patient registry","20-551","2026-01-09",{"date":251,"type":40},"2026-01-12",{"date":253,"type":40},"2020-12-22",{"date":255,"type":21},"2027-12",{"name":257,"class":87},"Memorial Sloan Kettering Cancer Center",{"id":259,"slug":260,"hasResults":11,"nctId":261,"briefTitle":262,"officialTitle":263,"acronym":264,"eligibilityCriteria":265,"healthyVolunteers":11,"sex":16,"minAge":212,"maxAge":266,"enrollmentInfo":267,"targetDuration":4,"studyType":22,"phases":269,"briefSummary":270,"conditions":271,"keywords":278,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":283,"lastUpdatePostDateStruct":284,"startDateStruct":286,"completionDateStruct":288,"leadSponsor":290,"locationsCount":88},"100581413","phase-2-io-combined-with-ai-as-first-line-treatment-for-patients-with-soft-tissue-sarcomatais-100581413","NCT06849986","IO Combined With AI as First-line Treatment for Patients With Soft Tissue Sarcoma(TAIS)","Tislelizumab Combined With Liposomal Doxorubicin and Ifosfamide as First-line Treatment for Patients With Specified Subtypes of Unresectable or Metastatic Soft Tissue Sarcoma: a Multi-center, Single-arm, Prospective Phase II Clinical Trial","TAIS","Inclusion Criteria:\n\n* Age 18 to 75 years, regardless of gender;\n* Patients with histopathologically confirmed undifferentiated sarcoma (except small round cell undifferentiated sarcoma), synovial sarcoma, angiosarcoma, fibrosarcoma, smooth muscle sarcoma, liposarcoma (except well differentiated liposarcoma), pleomorphic rhabdomyosarcoma, malignant peripheral nerve sheath meningiomas, desmoplastic small round cell tumor, not other specified (NOS), SMARCA4-deficient sarcoma, malignant phyllodes tumor of the breast, intimal sarcoma.\n* Patients with locally advanced disease that is not amenable to surgery\u002Fradiation therapy or with recurrent\u002Fmetastatic disease;\n* Eastern Cooperative Oncology Group (ECOG) performance status (PS) score of 0 to 1;\n* Expected survival of more than 3 months;\n* Within 7 days prior to screening (including day 7), laboratory test data requirements: neutrophil count ≥1.5×10⁹\u002FL, platelet count ≥90×10⁹\u002FL, hemoglobin ≥90g\u002FL (no blood transfusion within 14 days), serum total bilirubin ≤1.5 times the upper limit of normal (ULN); ALT and AST ≤2.5× ULN (≤5× ULN for patients with liver metastases); serum creatinine ≤1.5× ULN or creatinine clearance rate ≥50ml\u002Fmin;\n* Presence of measurable lesions according to RECIST 1.1 criteria;\n* The subject (or their legal representative\u002Fguardian) must sign an informed consent form, indicating that they understand the purpose of this study, are aware of the necessary procedures, and are willing to participate in this study.\n\nExclusion Criteria:\n\nAny of the following conditions will result in exclusion from the study:\n\n* Previous treatment for advanced soft tissue sarcoma, except for those who relapsed more than six months after adjuvant therapy with a cumulative dose of doxorubicin ≤300mg\u002Fm2;\n* Received any experimental or anti - tumor drugs within 4 weeks prior to enrollment;\n* Previously received any anti - PD - 1, anti - PD - L1, anti - PD - L2, anti - CD137, or anti - CTLA - 4 antibody treatment, or any other antibodies or drugs specifically targeting T - cell co - stimulation or checkpoint pathways;\n* History of other tumors within the past five years, except for cured cervical cancer or skin basal cell carcinoma; for patients with post - radiation sarcoma, another primary tumor must have no recurrence or metastasis;\n* Symptomatic brain or meningeal metastasis (unless the patient has been treated for more than 6 months, with negative imaging results within 4 weeks prior to enrollment, and stable tumor - related clinical symptoms at the time of enrollment);\n* Clinically significant active bleeding;\n* Pregnant or lactating women; women of childbearing potential who have not taken adequate contraceptive measures;\n* Alcohol abuse or drug addiction;\n* Patients with active autoimmune diseases or a history of such diseases that may recur (e.g., systemic lupus erythematosus, rheumatoid arthritis, inflammatory bowel disease, autoimmune thyroid disease, multiple sclerosis, vasculitis, glomerulonephritis, etc.), or those at high risk (such as patients who have undergone organ transplantation and require immunosuppressive therapy). Autoimmune hypothyroidism requiring only hormone replacement therapy or skin diseases not requiring systemic treatment are excluded;\n* Patients who need to receive systemic corticosteroids (equivalent to \\>10mg prednisone\u002Fday) within 14 days prior to enrollment or during the study, or those who require other immunosuppressive drug treatment. The use of topical or inhaled corticosteroids, or short - term (≤7 days) use of corticosteroids for prevention or treatment of non - autoimmune, non - frequent allergic diseases is excluded;\n* Failure of important organs or other severe diseases, including interstitial pneumonia, clinically significant coronary artery disease, cardiovascular disease, or myocardial infarction, congestive heart failure, unstable angina, symptomatic pericardial effusion, or unstable arrhythmia within 6 months prior to enrollment;\n* History of human immunodeficiency virus infection, or other acquired or congenital immune deficiency diseases, or history of organ transplantation or stem cell transplantation;\n* Patients with active chronic hepatitis B or active hepatitis C. HBV carriers, those with stable hepatitis B after drug treatment (DNA titer ≤10\\^3 copies\u002Fml), and those with cured hepatitis C (HCV RNA negative) are eligible for enrollment;\n* Severe neurological or psychiatric history; severe infection; active disseminated intravascular coagulation, or other concomitant diseases that, in the opinion of the investigator, seriously endanger the safety of the patient or affect the patient's ability to complete the study.","75 Years",{"count":268,"type":21},45,[25],"This study will enroll patients with specific subtypes of unresectable or metastatic soft tissue sarcoma, and will combine tislelizumab with the standard chemotherapy of liposomal doxorubicin and ifosfamide to initially explore the efficacy and safety.",[272,273,274,275,276,277,28],"Soft Tissue Sarcomas","Angiosarcoma","Fibrosarcoma","Leiomyosarcoma","Pleomorphic Liposarcoma","Malignant Peripheral Nerve Sheath Tumor (MPNST)",[279,280,281,282],"soft tissue sarcoma","tislelizumab","doxorubicin","ifosfamide","2026-01-07",{"date":285,"type":40},"2026-01-08",{"date":287,"type":40},"2025-02-25",{"date":289,"type":21},"2029-12-31",{"name":291,"class":87},"Fudan University",{"id":293,"slug":294,"hasResults":11,"nctId":295,"briefTitle":296,"officialTitle":297,"acronym":4,"eligibilityCriteria":298,"healthyVolunteers":11,"sex":16,"minAge":212,"maxAge":299,"enrollmentInfo":300,"targetDuration":4,"studyType":22,"phases":302,"briefSummary":303,"conditions":304,"keywords":4,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":309,"lastUpdatePostDateStruct":310,"startDateStruct":312,"completionDateStruct":314,"leadSponsor":316,"locationsCount":88},"100537386","phase-2-masct-i-combined-with-doxorubicin-and-ifosfamide-for-first-line-treatment-of-advanced-soft-tissue-sarcoma-100537386","NCT06277154","MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment of Advanced Soft Tissue Sarcoma","A Phase II Study Evaluating the Safety and Efficacy of MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment in Patients With Advanced Soft Tissue Sarcoma","Inclusion Criteria:\n\n1. Age≥18 years and≤70 years;\n2. According to WHO Classification of Tumours, 5th Edition, Volume 3: Soft Tissue and Bone Tumours, histopathologically or cytologically confirmed unresectable locally advanced or metastatic soft tissue sarcomas, including leiomyosarcoma, liposarcoma, synovial sarcoma, angiosarcoma, undifferentiated pleomorphic sarcoma, epithelioid sarcoma, malignant peripheral nerve sheath tumors, fibrosarcoma, pleomorphic rhabdomyosarcoma, endometrial stromal sarcoma, desmoplastic small round cell tumor.\n3. No previous treatment with systematic chemotherapy or targeted therapy for advanced soft tissue sarcomas or whose disease progressed after 6months of the end of neoadjuvant or adjuvant therapy.\n4. At least one measurable and assessable lesion defined by RECIST 1.1;\n5. ECOG performance status of 0-1;\n6. Estimated life expectancy≥6 months;\n7. Pulmonary function is basically normal;\n8. Subjects with organ function as defined below (any blood components and growth factors are not allowed within 14 days before apheresis): a) Hemoglobin ≥90g\u002FL; b) Leukocyte≥3.5x10\\^9\u002FL; c) The absolute neutrophil count (ANC)\\>1.5x10\\^9\u002FL; d) Platelet≥100x10\\^9\u002FL; e) ALT, AST≤2.5 ULN (Upper Limit of Normal), ALT, AST≤5 ULN for liver metastases; f) ALP≤2.5 ULN; g) Serum total bilirubin≤1.5 ULN; Patients with Gilbert's syndromes (persistent or repeated hyperbilirubinemia \\[mainly unconjugated bilirubin\\], in the absence of evidence of hemolysis or liver disease), are allowed to enroll with investigator's agreement; h) Serum urea nitrogen or urea and creatinine≤2.5 ULN; i) Serum albumin≥35g\u002FL; j) PT, APTT, INR≤1.5 ULN (without anticoagulation treatment);\n9. Obtain the written informed consent of the patient\u002Flegal representative;\n10. Subjects with potential fertility must agree to use effective contraceptive measure during and within 6 months after the treatment period. HCG test for female with potential fertility must be negative before the study was included.\n\nExclusion Criteria:\n\n1. Previous treatment with targeted therapy, radiotherapy (radiotherapy to non-target lesions or disease progressed after radiotherapy could be included.) or other antineoplastic drugs such as anlotinib, gemcitabine, within 4 weeks before randomization, or have received Chinese medicine or proprietary Chinese medicine for anti-tumor treatment within 2 weeks before randomization.\n2. Highly differentiated liposarcoma, malignant perivascular epithelioid tumor, protuberant cutaneous fibrosarcoma, extraosseous osteosarcoma, solitary fibroma\u002Fhemangiopericytoma, acinous soft tissue sarcoma, extraosseous myxoid chondrosarcoma, gastrointestinal stromal tumor, invasive fibroma, renal angiomyolipoma, malignant mesothelioma, clear cell sarcoma, Ewing's sarcoma, etc., which are not suitable for Doxorubicin+ Ifosfamide (AI) treatment.\n3. Previous treatment with anthracyclines or anthraquinones and whose cumulative dose exceeds equivalent 200mg\u002Fm2 doxorubicin.\n4. Previous treatment with MASCT, or have received other cellular immunotherapy or anti-PD-1, anti-PD-L1 antibody therapy in the past 1 year.\n5. Use of immunosuppressive agents or systemic or inhaled local hormones (exceeding 10mg\u002Fday prednisone or its equivalent) and were still using them within 2 weeks before randomization.\n6. Use of immunomodulators and were still using them within 2 weeks before randomization.\n7. Allergic to sodium citrate or human albumin.\n8. Subjects with uncontrolled pleural effusion and abdominal effusion requiring repeated drainage and with moderate or higher volume of pericardial effusion.\n9. Have known active central nervous system (CNS) or meningeal metastases. Subjects with previously treated brain metastases may participate provided they are stable based on the following: 1) any neurologic symptoms have returned to baseline at least 2 weeks before randomization, 2) no requirement for steroids at least 2 weeks before randomization or receiving low-dose of steroids (Not exceeding 10mg\u002Fday prednisone or its equivalent).\n10. Have any active autoimmune disease or history of autoimmune disease.\n11. Subjects with active tuberculosis.\n12. Subjects were infected with hepatitis B virus, hepatitis C virus or HIV, or syphilis.\n13. Severe cardiovascular disease, such as: (1) complete left bundle branch block or III atrioventricular block; (2) history of myocardial infarction, angioplasty, coronary artery bypass graft; (3) prolonged QT\u002FQTc interval at baseline (male\\>450ms, female \\>480ms); (4) LVEF≤50%; (5) heart failure of NYHA class 2 or higher; (6) poorly controlled hypertension (BP≥150\u002F95 mmHg, despite optimal medical treatment); (7) cardiomyopathy or severe arrhythmia and may have impact on the study based on investigator's judgement.\n14. Subjects with history of thrombus or experienced a cerebrovascular accident within 6 months before randomization;\n15. Other malignant tumors (except cured skin basal cell carcinoma, prostate carcinoma in situ and cervical carcinoma in situ) in the past 5 years;\n16. Known history of organ transplantation or ready to receive an organ transplantation;\n17. Subjects who have undergone major surgery or traumatic injury within 4 weeks before randomization;\n18. Those who have a history of alcohol dependence, psychotropic substance abuse and cannot abstain or have mental disorders.\n19. Surgery for soft tissue sarcoma is planned during the study.\n20. Subjects have participated in another investigational trial within 4 weeks before randomization.\n21. Any condition that the investigator considers to be prejudicial to the subject or to the subject's inability to meet or perform the study requirements exists.","70 Years",{"count":301,"type":21},148,[25],"This study will evaluate the safety and efficacy of MASCT-I combined with Doxorubicin and Ifosfamide for first-line treatment in patients with advanced soft tissue sarcoma.",[275,305,65,273,306,71,132,274,307,308,28],"Liposarcoma","Undifferentiated Pleomorphic Sarcoma","Pleomorphic Rhabdomyosarcoma","Endometrial Stromal Sarcoma","2025-07-20",{"date":311,"type":40},"2025-07-24",{"date":313,"type":40},"2024-02-21",{"date":315,"type":21},"2027-02",{"name":317,"class":318},"HRYZ Biotech Co.","INDUSTRY",{"id":320,"slug":321,"hasResults":11,"nctId":322,"briefTitle":323,"officialTitle":324,"acronym":325,"eligibilityCriteria":326,"healthyVolunteers":11,"sex":16,"minAge":327,"maxAge":328,"enrollmentInfo":329,"targetDuration":4,"studyType":22,"phases":330,"briefSummary":331,"conditions":332,"keywords":333,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":344,"lastUpdatePostDateStruct":345,"startDateStruct":347,"completionDateStruct":349,"leadSponsor":351,"locationsCount":353},"100551151","phase-2-pasireotide-as-maintenance-treatment-in-synovial-sarcoma-and-desmoplastic-small-round-cell-tumor-100551151","NCT06456359","Pasireotide as Maintenance Treatment in Synovial Sarcoma and Desmoplastic Small Round Cell Tumor","Pasireotide as Maintenance Treatment With Monthly Deep Intramuscular Injection in SSTR2\u002F3\u002F5-Expressing Synovial Sarcoma and Desmoplastic Small Round Cell Tumor","PAMSARC","Inclusion Criteria:\n\nPatients meeting all of the following criteria are considered for enrolment into the trial:\n\n1. Reference pathological proven diagnosis of DSRCT in any stage; or Reference pathological proven diagnosis of SySa, IRS III, metastatic or relapsed disease\n2. High SSTR2\u002F3\u002F5 mRNA expression, as determined by RNA sequencing in the DKFZ\u002FNCT\u002FDKTK MASTER (ClinicalTrials.gov ID: NCT05852522) or INFORM programs.\n3. Stable disease, partial or complete response after completion of standard treatment\n4. Age from 13 to 50 years\n5. For patients (≥16 years): Karnofsky-Index ≥ 80% For patients (\\\u003C16 years): Lansky-Index ≥ 80%\n6. No curative treatment option\n7. Bodyweight ≥ 30kg and BSA ≥ 1.1m²\n8. Time from last chemotherapy (at least 2 chemotherapy cycles) to enrollment \\\u003C8 weeks\n9. Ability of patient to understand character and individual consequences of the clinical trial\n10. Written informed consent (for individuals \\\u003C18 years of age an ICF for adolescents and their parents is needed)\n11. For women of childbearing potential negative urine pregnancy test at screening as well as highly effective forms of contraception have to be in place thereafter\n\n    * Evidence of childbearing potential is defined as fertile, following menarche and until becoming post-menopausal unless permanently sterile\n    * Postmenopausal or evidence of non-childbearing status is defined as:\n\n      * Amenorrhea for 1 year or more without an alternative medical cause following cessation of exogenous hormonal treatments plus follicle stimulating hormone (FSH) levels in the postmenopausal range in women not using hormonal contraception or hormonal replacement therapy.\n      * Chemotherapy-induced menopause\n      * Surgical sterilization (bilateral oophorectomy, bilateral salpingectomy, total hysterectomy or tubal ligation at least 6 weeks before IMP treatment)\n    * A man is considered fertile after puberty unless permanently sterile by bilateral orchidectomy\n12. Female patients of childbearing potential and male patients with partners of childbearing potential who are sexually active must agree to the use of two forms of contraception in combination (male condom and one highly effective method). These should be started immediately after signing the informed consent form and continued throughout the period of study treatment plus 3 months for female and male patients . Male patients should refrain from fathering a child or donating sperm during the trial and for at least 3 months following the last dose.\n13. Adequate bone marrow, renal, and hepatic function defined by laboratory tests within 14 days prior to study treatment:\n\n    * Hemoglobin ≥ 10 g\u002Fdl\n    * Neutrophil count ≥ 1,500\u002Fmm3\n    * Platelet count ≥ 100,000\u002Fµl\n    * Bilirubin ≤ 1.5 x upper limit of normal (ULN)\n    * ALT and AST ≤ 2.5 x ULN\n    * Alkaline phosphatase ≤ 2.5 x ULN\n    * PT-INR\u002FPTT ≤ 1.5 x ULN\n    * Albumin ≥ 25 g\u002Fl\n    * Creatine kinase ≤ 2.5 x ULN\n    * Serum creatinine \\\u003C or = 1.5 mg\u002Fdl or creatinine clearance = or \\> 51 ml\u002Fmin (calculation according to Crockroft-Gault)\n\nExclusion Criteria:\n\nPatients presenting with any of the following criteria are not included in the trial:\n\n1. History of hypersensitivity to the investigational medicinal product or to any drug with similar chemical structure or to any excipient present in the pharmaceutical form of the investigational medicinal product\n2. Concurrent or previous treatment within 30 days in another interventional clinical trial \u002F Participation in other ongoing clinical trials.\n3. Uncontrolled concurrent disease, in particular diabetes mellitus\n4. Bleeding disorder\n5. Therapeutic anticoagulation which cannot be paused temporarily in order to ensure safe intramuscular injection\n6. Is taking or requiring any of the prohibited medication listed in Table 5 (6.4.2)\n7. Heart rate at rest \\\u003C 60\u002Fmin\n8. fasting glucose level \\> 110mg\u002Fdl\n9. Severe neurologic or psychiatric disorder\n10. Pregnancy\u002Flactation\n11. Prior treatment with somatostatin analog\n\n    \\-","13 Years","50 Years",{"count":48,"type":21},[25],"PAMSARC is a non-commercial interventional Phase 2 clinical trial of academic research institutions, with its primary goal being to improve medical treatment of fusion driven Desmoplastic small round cell tumor (DSRCT) and Synovial sarcoma (SySa) in young adults and adolsecents with male predominance.\n\nCurrent management of DSRCT and SySa includes chemotherapy, radiation and aggressive cytoreductive surgery. Despite advances in multimodal therapy, outcomes remain poor with frequent disease recurrence and very limited options for patients with advanced disease.\n\nSelected somatostatin receptor (SSTR) family members, i.e., SSTR2, SSTR3 and SSTR5, are frequently overexpressed in DSRCT and SySa, providing the rationale for treatment with somatostatin analogues (SSA).\n\nPasireotide is a SSA with high affinity for SSTR1, -2, -3, and -5 and is approved for the treatment of Cushing's disease and acromegaly and has also shown activity in other cancers. In patients with advanced stage DSRCT and SySa, conventional chemotherapeutic approaches frequently lead to disease response, however, the duration of progression-free time after chemotherapy is short. The targeted approach with pasireotide after initial intensive multimodal treatment may have the potential to significantly improve outcome.",[28,65],[334,335,336,337,338,339,340,341,342,343],"SSTR","somatostatin receptor","SSTR2, SSTR3, SSTR5","somatostatin analog","pasireotide","fusion-gene driven","SS18-SSX1, SS18-SSX2, SS18-SSX4","EWSR1-WT1","extremely rare, aggressive sarcoma","maintaince therapy","2025-01-09",{"date":346,"type":40},"2025-01-13",{"date":348,"type":40},"2024-12-19",{"date":350,"type":21},"2028-10",{"name":352,"class":87},"University Hospital Heidelberg",4]