[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"nf2-related-schwannomatosis\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:nf2-related-schwannomatosis":30},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,3,0,[8,45,70],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":13,"acronym":14,"eligibilityCriteria":15,"healthyVolunteers":11,"sex":16,"minAge":4,"maxAge":4,"enrollmentInfo":17,"targetDuration":4,"studyType":20,"phases":21,"briefSummary":23,"conditions":24,"keywords":4,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":33,"lastUpdatePostDateStruct":34,"startDateStruct":37,"completionDateStruct":39,"leadSponsor":41,"locationsCount":44},"100647902","phase-2-platform-research-for-innovative-medicines-in-nf2-swn-prime-nf2-100647902",false,"NCT07713745","Platform Research for Innovative Medicines in NF2-SWN (PRIME-NF2)","PRIME-NF2","Eligibility Specific For MASTER STUDY\n\nInclusion Criteria:\n\nSubjects must satisfy all of the following criteria to be enrolled into the main study natural history observation cohort:\n\n(1) Must meet the 2022 International Consensus Criteria for NF2-SWN, defined by having at least one of the following:\n\n1. Bilateral vestibular schwannomas (VS)\n2. An identical NF2 pathogenic variant in at least 2 anatomically distinct NF2-related tumors (schwannoma, meningioma, and\u002For ependymoma). (Note: if the variant allele fraction (VAF) in unaffected tissues such as blood is clearly \\\u003C50%, the diagnosis is mosaic NF2-related schwannomatosis)\n3. Either 2 major or 1 major and 2 minor criteria as described in the following:\n\nMajor criteria:\n\n* Unilateral VS\n* First-degree relative other than sibling with NF2-related schwannomatosis\n* 2 or more meningiomas (Note: single meningioma qualifies as minor criteria).\n* NF2 pathogenic variant in an unaffected tissue such as blood (Note: if the VAF is clearly \\\u003C50%, the diagnosis is mosaic NF2-related schwannomatosis)\n\nMinor criteria:\n\nCan count \\>1 of a type (eg, 2 distinct schwannomas would count as 2 minor criteria)\n\n* Ependymoma, meningioma (Note: multiple meningiomas qualify as a major criteria), schwannoma (Note: if the major criterion is unilateral VS, at least 1 schwannoma must be dermal in location) Can count only once (eg, bilateral cortical cataracts count as a single minor criterion)\n* Juvenile subcapsular or cortical cataract, retinal hamartoma, epiretinal membrane in a person aged \\\u003C40 years, meningioma\n\n  (2) Presence of at least one evaluable lesion: Vestibular schwannoma, meningioma, or non-vestibular schwannoma: clearly identifiable lesion on contrast-enhanced T1-weighted MRI; Ependymoma: clearly identifiable lesion on contrast-enhanced T1-weighted or T2\u002FFLAIR sequences; (3) Expected ability to complete at least 12 months of follow-up assessments; (4) Ability to understand and voluntarily sign a written informed consent form, or a legally authorized guardian signs the informed consent form together ; (5) Sub-study-specific criteria (for intervention arms only): If the subject intends to enter an interventional sub-study, in addition to meeting the above main study criteria, the subject must also satisfy the specific inclusion criteria specified in that sub-study protocol (e.g., organ function, prior treatment restrictions, washout periods, etc.), as determined by the drug characteristics.\n\nExclusion Criteria:\n\nSubjects meeting any of the following criteria will not be permitted to enter the main study:\n\n1. Coexisting other genetic syndromes that may cause multiple intracranial tumors (e.g., SMARCB1\u002FLZTR1-related schwannomatosis, Cowden syndrome);\n2. Expected survival \\\u003C12 months;\n3. Presence of severe psychiatric disorders or cognitive impairment that precludes cooperation with imaging or hearing assessments;\n4. Extreme social or geographic factors that, in the investigator's judgment, may impede follow-up for more than 12 months;\n5. Sub-study-specific exclusion criteria (for intervention arms only): If the subject intends to enter an interventional sub-study, the subject must also satisfy the specific exclusion criteria specified in that sub-study protocol (e.g., specific organ dysfunction, active infection, pregnancy, etc.).","ALL",{"count":18,"type":19},200,"ESTIMATED","INTERVENTIONAL",[22],"PHASE2","This is an adaptive platform-basket trial that aims to evaluate the safety and efficacy of multiple novel agents and combination therapies in patients with NF2-related schwannomatosis (NF2-SWN). The study employs a basket design to assess treatment responses across four tumor types commonly associated with NF2-SWN: vestibular schwannomas, non-vestibular schwannomas, meningiomas, and ependymomas.\n\nA shared natural history observational cohort, receiving routine clinical follow-up without investigational treatment, serves as a common control for all substudies. The adaptive platform enables the dynamic addition or closure of substudies based on interim analyses, thereby optimizing trial efficiency.\n\nEligible patients who meet the master protocol criteria and satisfy substudy-specific safety requirements will be assigned to receive the corresponding intervention. Currently open substudies include:\n\n* Substudy A: Selumetinib\n* Substudy B: Luvometinib plus Serplulimab",[25,26,27,28,29,30,31],"Neurofibromatosis Type 2","Vestibular Schwannoma","Non-vestibular Schwannoma","Meningioma","Ependymoma","NF2-related Schwannomatosis","NF2","NOT_YET_RECRUITING","2026-07-14",{"date":35,"type":36},"2026-07-20","ACTUAL",{"date":38,"type":19},"2026-07-18",{"date":40,"type":19},"2036-12-31",{"name":42,"class":43},"Beijing Tiantan Hospital","OTHER",5,{"id":46,"slug":47,"hasResults":11,"nctId":48,"briefTitle":49,"officialTitle":50,"acronym":51,"eligibilityCriteria":52,"healthyVolunteers":11,"sex":16,"minAge":53,"maxAge":4,"enrollmentInfo":54,"targetDuration":4,"studyType":20,"phases":56,"briefSummary":57,"conditions":58,"keywords":59,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":33,"lastUpdatePostDateStruct":62,"startDateStruct":64,"completionDateStruct":66,"leadSponsor":68,"locationsCount":69},"100647361","phase-2-selumetinib-for-nf2-related-schwannomatosis-100647361","NCT07707947","Selumetinib for NF2-Related Schwannomatosis","A Single-Center, Single-Arm, Phase II Study to Explore the Efficacy and Safety of MEK1\u002F2 Inhibitor (MEKi) Selumetinib in the Treatment of Patients With NF2-Related Schwannomatosis","ASSIST","Inclusion Criteria:\n\n1. Patients must have a pathogenic variant in the NF2 gene (either in the germline or in two NF2-related tumors)OR a confirmed diagnosis of NF2 by fulfilling National Institute of Health (NIH)criteria or Manchester criteria:\n\n   The genetic test report should be issued by companies or hospitals with corresponding qualifications.\n\n   The NIH criteria includes presence of:\n   * Bilateral vestibular schwannomas, OR\n   * First-degree relative with NF2 and EITHER unilateral eighth nerve mass OR two of the following: neurofibroma, meningioma, glioma, schwannoma, juvenile posterior subcapsular lenticular opacity.\n\n   The Manchester criteria includes presence of:\n   * Bilateral vestibular schwannomas, OR\n   * First-degree relative with NF2 and EITHER unilateral eighth nerve mass OR two of the following: neurofibroma, meningioma, glioma, schwannoma, juvenile posterior subcapsular lenticular opacity, OR\n   * Unilateral vestibular schwannoma AND any two of: neurofibroma, meningioma, glioma, schwannoma, juvenile posterior subcapsular lenticular opacity,OR\n   * Multiple meningiomas (two or more)AND unilateral vestibular schwannoma OR\n   * any two of: schwannoma, glioma, neurofibroma, cataract.\n2. Subjects must have ≥1 measurable target vestibular schwannoma meeting all of the following conditions:\n\n   * Measurable on MRI with a minimum diameter ≥3 mm;\n   * Evidence of radiographic progression within the past 36 months according to REiNS criteria, OR documented clinical progression attributable to the target tumor (e.g., hearing decline, cranial nerve dysfunction).\n   * Subjects whose Word Recognition Score (WRS)ranging from 50%to 88%at the side of target vestibular schwannoma.\n3. The target tumor must be considered not amenable to surgery due to:\n\n   * High surgical risk (e.g., risk of neurological deficit), OR\n   * Patient refusal of surgery after adequate medical counseling.\n4. Male or female subjects aged ≥3 years at the time of informed consent.\n5. Adequate functional status\n\n   Subjects aged ≥16 years:\n   * Karnofsky Performance Status ≥70,OR\n   * ECOG Performance Status 0-1\n\n   Subjects aged \\\u003C16 years:\n   * Lansky Performance Status ≥70\n   * Adequate organ and bone marrow function\n6. Laboratory values obtained within 28 days prior to enrollment must meet the following:\n\n   1. Hematologic function\n\n      * Hemoglobin ≥9.0 g\u002FdL\n      * Absolute neutrophil count (ANC)≥1.5 ×10⁹\u002FL\n      * Platelet count ≥100 ×10⁹\u002FL\n   2. Hepatic function\n\n      * AST and ALT ≤2.5 ×ULN (≤2.0 ×ULN for pediatric subjects)\n      * Total bilirubin ≤ 1.5 ×ULN (≤3 ×ULN in subjects with documented Gilbert's syndrome)\n   3. Renal function\n\n      * Serum creatinine ≤1.5 ×ULN, OR\n      * Creatinine clearance ≥60 mL\u002Fmin\u002F1 .73 m²(age-and BSA-adjusted)\n   4. Cardiac function\n\n      * Left ventricular ejection fraction (LVEF)≥50%by echocardiography\n      * No clinically significant uncontrolled cardiac disease\n   5. Pulmonary function • Peripheral oxygen saturation ≥92%on room air\n7. Reproductive and contraception requirements\n\n   1. Females of childbearing potential\n\n      * Negative serum or urine pregnancy test prior to enrollment\n      * Must be postmenopausal ≥12 months, surgically sterile, or using a highly effective method of contraception throughout the study and for 3 months after the last dose\n      * Acceptable methods include:\n\n        * Hormonal contraception\n        * Intrauterine device (IUD)\n        * Long-acting reversible contraception\n        * Tubal ligation\n      * Oral contraception alone must be combined with a barrier method\n   2. Males • Must be surgically sterile or agree to use barrier contraception with permicide during treatment and for 3 months after the last dose.\n8. Subjects must be able to swallow selumetinib capsules intact.\n9. Written informed consent must be obtained:\n\n   * From the subject if capable of providing consent;\n   * From a legally acceptable representative for minors or subjects lacking full decision-making capacity.\n\nExclusion Criteria:\n\n1. Concurrent involvement in study conduct:the subject is an employee of the Sponsor, Investigator, or study site who is directly involved in the planning, conduct, or management of this clinical study.\n2. Participation in another interventional clinical trial with an investigational medicinal product, or receipt of an investigational agent within 28 days (or 5 half-lives if known and longer)prior to first dose.\n3. Prior anti-cancer systemic therapies or prior radiotherapy that, in the investigator's judgment, would confound safety or efficacy assessment, unless completed ≥28 days prior to first dose (longer washout required for agents with prolonged biologic effect as specified in protocol appendix). Subjects who received prior local therapy (surgery or localized radiotherapy)are eligible if recovery is complete and target lesion remains measurable.\n4. Evidence or high suspicion of malignant peripheral nerve sheath tumor (MPNST)or other active malignancy requiring systemic therapy (past malignancy is allowed only if disease-free for ≥2 years, except for adequately treated basal cell carcinoma or in situ carcinoma).\n5. Significant cardiac disease or ECG abnormalities:\n\n   * Resting QTcF \\>470 ms (adults)\\[\\>450 ms for pediatric thresholds as specified in protocol appendix\\], or clinically significant baseline prolongation per investigator\u002Fmedical monitor.\n   * Clinically significant arrhythmia (symptomatic ventricular tachycardia, sustained ventricular tachycardia, uncontrolled atrial fibrillation). Subjects with well controlled atrial fibrillation may be considered after Medical Monitor review.\n   * Acute coronary syndrome within 6 months, unstable angina, symptomatic congestive heart failure NYHA class II-IV, or LVEF below institutional lower limit of normal (LLN)or \\\u003C50%.\n6. Uncontrolled hypertension:systolic ≥140 mmHg or diastolic ≥90 mmHg despite optimal therapy (adult criteria);for pediatric subjects use age\u002Fheight\u002Fgender percentiles per protocol appendix.\n7. Severe hepatic or renal impairment -e.g.,AST\u002FALT \\>5 ×ULN (or per protocol specified threshold for severe impairment), total bilirubin \\>3 ×ULN (unless Gilbert's syndrome).(See inclusion for minimum acceptable labs;exclude those with more severe dysfunction.)\n8. Ophthalmologic conditions:current or prior history of MEK-associated retinopathy \u002F central serous retinopathy \u002Fretinal pigment epithelial detachment \u002Fretinal vein occlusion, or any active ocular condition judged by the investigator\u002Fophthalmologist to increase the risk of serious ocular adverse events (e.g., uncontrolled glaucoma with elevated IOP and meaningful vision at risk). Subjects with stable, chronic ophthalmic findings that are not expected to worsen with MEK inhibition may be eligible after ophthalmology clearance.\n9. Known hypersensitivity to selumetinib or any excipients.\n10. Active, uncontrolled infection including:\n\n    * Positive HIV test (known HIV infection with uncontrolled disease or on unstable antiretroviral therapy that interacts with study drug)-no evidence AIDS and no contraindicating ART may be considered after Medical Monitor review.\n    * Active hepatitis B or C infection (HBsAg positive or HCV RNA positive). Subjects with resolved HBV (HBsAg negative, anti-HBc positive)may be eligible per local hepatology guidance and prophylaxis plan.\n11. Concomitant medications that:\n\n    * Are known to prolong QT interval and cannot be safely discontinued;OR\n    * Are strong CYP3A4 or CYP2C19 inducers\u002Finhibitors that cannot be stopped and would significantly alter selumetinib exposure (refer to protocol drug-interaction appendix for permitted\u002Fforbidden lists).\n12. Gastrointestinal conditions that preclude reliable oral absorption (e.g., severe malabsorption, short bowel syndrome, recent (within 6 months)major intestinal resection)or inability to swallow intact capsules.\n13. Conditions requiring urgent neurosurgical intervention (e.g., symptomatic hydrocephalus requiring immediate shunting, life-threatening brainstem compression)-subjects requiring emergent neurosurgery are not eligible until stabilized and reevaluated.\n14. Prior organ transplantation (allogeneic)or ongoing immunosuppression that would confound safety assessment.\n15. Concomitant vitamin E†or other agents judged to have potential interaction with the study drug if they cannot be discontinued per protocol (e.g., vitamin E to be stopped ≥7 days before first dose if required by protocol).\n16. Pregnancy or breastfeeding at screening (positive pregnancy test). Female subjects of childbearing potential who are unwilling\u002Funable to use acceptable contraception during the study and for the duration specified in the contraceptive guidance (see protocol)are excluded.\n17. Any other clinically significant medical, psychiatric, or social condition that, in the opinion of the investigator or Medical Monitor, would compromise subject safety or protocol compliance (including inability to undergo MRI, when MRI is required for tumor assessment).","3 Years",{"count":55,"type":19},20,[22],"The goal of this clinical trial is to evaluate the efficacy and safety of the MEK1\u002F2 inhibitor selumetinib in treating patients with neurofibromatosis type 2-related schwannomatosis (NF2-SWN), including both adults and children with inoperable or progressive tumors.",[25,31,30,28,29,26],[60,61],"NF2-SWN","Selumetinib",{"date":63,"type":36},"2026-07-16",{"date":65,"type":19},"2026-07-15",{"date":67,"type":19},"2028-12-31",{"name":42,"class":43},1,{"id":71,"slug":72,"hasResults":11,"nctId":73,"briefTitle":74,"officialTitle":75,"acronym":4,"eligibilityCriteria":76,"healthyVolunteers":11,"sex":16,"minAge":77,"maxAge":4,"enrollmentInfo":78,"targetDuration":4,"studyType":20,"phases":80,"briefSummary":82,"conditions":83,"keywords":4,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":33,"lastUpdatePostDateStruct":84,"startDateStruct":85,"completionDateStruct":87,"leadSponsor":89,"locationsCount":44},"100647329","phase-1-luvometinib-in-combination-with-serplulimab-for-nf2-related-tumors-100647329","NCT07708285","Luvometinib in Combination With Serplulimab for NF2-Related Tumors","A Multicenter, Open-Label Study of Luvometinib in Combination With Serplulimab for the Treatment of NF2-Related Tumors","Inclusion Criteria:\n\n1. Participants must meet the diagnostic criteria for NF2-SWN.\n2. Presence of at least one measurable target tumor, either vestibular schwannoma or meningioma, with MRI-documented volumetric progression within the past 36 months or clinical symptom progression related to the target tumor.\n3. The target tumor is considered unsuitable for surgery because of high risk.\n4. Age \\>=18 years at the time of screening.\n5. KPS \\>=70 or ECOG performance status 0 or 1.\n6. Adequate organ function based on laboratory tests obtained within 14 days before screening.\n7. Participant must be able to understand the study and voluntarily sign informed consent.\n\nExclusion Criteria:\n\n1. Pregnant, planning to become pregnant, or currently breastfeeding.\n2. Participation in another interventional clinical trial within the past 4 weeks, or radiation therapy to target lesion(s) within the past 3 years.\n3. Severe adverse reactions or intolerable toxicity from prior immune checkpoint inhibitors or MEK inhibitors.\n4. Concomitant active malignancies, uncontrolled infections, or active autoimmune diseases.\n5. Severe cardiac, hepatic, renal, gastrointestinal, or ophthalmic diseases.\n6. Any other factor that may compromise participant safety or study integrity.","18 Years",{"count":79,"type":19},30,[81,22],"PHASE1","This is an investigator-initiated, exploratory, multicenter, open-label, single-arm clinical trial and a substudy of the Platform Research for Innovative Medicines in NF2-SWN (PRIME-NF2). The study aims to evaluate the safety, tolerability, and preliminary efficacy of luvometinib in combination with serplulimab in patients with NF2-related schwannomatosis (NF2-SWN) with progressive tumors.",[30,31,25,26,28],{"date":63,"type":36},{"date":86,"type":19},"2026-07-31",{"date":88,"type":19},"2029-12-31",{"name":42,"class":43}]