NCT04374305

Brief Summary

This is a multi-arm phase II platform-basket screening study designed to test multiple experimental therapies simultaneously in patients with NF2-related schwannomatosis (NF2-SWN, formerly known as neurofibromatosis type 2) with associated progressive tumors of vestibular schwannomas (VS), non-vestibular schwannomas (non-VS), meningiomas, and ependymomas. This Master Study is being conducted as a "basket" study that may allow people with multiple tumor types associated with NF2-SWN to receive new drugs throughout this study. Embedded within the Master Study are individual drug substudies.

  • Investigational Drug Sub-study A: Brigatinib
  • Investigational Drug Sub-study B: Neratinib
  • Investigational Drug Sub-study C: Retifanlimab plus bevacizumab

Trial Health

77
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
109

participants targeted

Target at P50-P75 for phase_2

Timeline
55mo left

Started Jun 2020

Longer than P75 for phase_2

Geographic Reach
1 country

6 active sites

Status
recruiting

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Progress56%
Jun 2020Dec 2030

First Submitted

Initial submission to the registry

April 30, 2020

Completed
5 days until next milestone

First Posted

Study publicly available on registry

May 5, 2020

Completed
2 months until next milestone

Study Start

First participant enrolled

June 20, 2020

Completed
9.5 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

December 1, 2029

Expected
1 year until next milestone

Study Completion

Last participant's last visit for all outcomes

December 1, 2030

Last Updated

May 6, 2026

Status Verified

May 1, 2026

Enrollment Period

9.5 years

First QC Date

April 30, 2020

Last Update Submit

May 4, 2026

Conditions

Keywords

neurofibromatosis type 2 (NF2)vestibular schwannomas (VS)non-vestibular schwannomas (non-VS)meningiomaependymoma

Outcome Measures

Primary Outcomes (1)

  • Radiographic response rate (for each drug substudy)

    Radiographic response rates in target tumors according to tumor-associated criteria: * VS, non-VS, and meningiomas: Dombi criteria (2013) * Ependymomas: RECIST 1.12

    2 years

Secondary Outcomes (1)

  • Incidence of Treatment-Emergent Adverse Events [Safety and Tolerability

    2 years

Study Arms (3)

Sub-study A (brigatinib) - CLOSED TO ENROLLMENT

EXPERIMENTAL

Subjects treated in this arm will receive brigatinib 90 mg by mouth daily for 7 days and then increased to 180 mg by mouth daily if the drug is tolerated.

Drug: Brigatinib

Sub-study B (neratinib)

EXPERIMENTAL

The first three participants treated in this arm will receive neratinib 200 mg mg by mouth daily. If these participants tolerate the medication well, subsequent participants will receive neratinib 240 mg by mouth daily.

Drug: Neratinib

Experimental: sub-study C (retifanlimab plus bevacizumab)

EXPERIMENTAL

Subjects on this arm will receive retifanlimab by IV infusion at a dose of 375 mg every 3 weeks on a continuous schedule for 48 weeks. Subjects will also receive bevacizumab by IV infusion at a dose of 7.5 mg/kg every 3 weeks on an intermittent schedule. One cycle last 42 days and the study will last for 52 total weeks (48 treatment weeks and 4 follow-up weeks).

Drug: RetifanlimabDrug: Bevacizumab

Interventions

Every 3 weeks by IV per predetermined dose in protocol.

Experimental: sub-study C (retifanlimab plus bevacizumab)

Every 3 weeks by IV per predetermined dose in protocol.

Also known as: Avastin, MVASI
Experimental: sub-study C (retifanlimab plus bevacizumab)

Oral daily per predetermined dosage per protocol.

Also known as: Alunbrig
Sub-study A (brigatinib) - CLOSED TO ENROLLMENT

Oral daily per predetermined dosage per protocol.

Also known as: Nerlynx
Sub-study B (neratinib)

Eligibility Criteria

Age12 Years+
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

  • \- 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:
  • The NIH criteria includes presence of:
  • Bilateral vestibular schwannomas, OR
  • 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.
  • The Manchester criteria includes presence of:
  • Bilateral vestibular schwannomas, OR
  • 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
  • Unilateral vestibular schwannoma AND any two of: neurofibroma, meningioma, glioma, schwannoma, juvenile posterior subcapsular lenticular opacity, OR
  • Multiple meningiomas (two or more) AND unilateral vestibular schwannoma OR any two of: schwannoma, glioma, neurofibroma, cataract.
  • Subjects must have a target NF2-related tumor (VS, non-VS, meningioma, or ependymoma) with documented radiographic progression within the preceding 36 months of Master Study registration defined as either:
  • at least 20% increase in volume of enhancing tumor
  • at least 2 mm increase in greatest linear dimension of enhancing tumor
  • Participants must have measurable disease, defined as:
  • VS, non-VS, or meningioma target lesions that can be accurately measured as at least 1 ml by volumetric MRI scan or in at least one dimension as ≥10 mm with conventional MRI scan. See protocol for the evaluation of measurable disease
  • Ependymoma target lesions measurable linearly.
  • +3 more criteria

You may not qualify if:

  • Participants who have had chemotherapy within a minimum of 4 weeks prior to Master Study registration (or a minimum of 5 half-lives and resolution to baseline of toxicities unless there are irreversible toxicities from prior drug that do not influence risk of next drug).
  • Participants who have received radiation to the target tumor within the last 3 years prior to Master study registration.
  • Participants who are receiving any other investigational agents.
  • Participants with target or non-target nervous system tumors that, in the opinion of the treating investigator, are likely to require active treatment (including surgery) within 6 months of registration to the Master Study.
  • History of a different malignancy, unless (a) have been disease-free for at least 2 years and are deemed by the treating investigator to be at low risk for recurrence of that malignancy.
  • Uncontrolled intercurrent illness including, but not limited to, ongoing or active infection, symptomatic congestive heart failure, unstable angina pectoris, cardiac arrhythmia, or psychiatric illness/social situations that would limit compliance with study requirements.
  • Pregnant women are excluded from this study because the experimental agents may have the potential for teratogenic or abortifacient effects. Because there is an unknown but potential risk for adverse events in nursing infants secondary to treatment of the mother with these experimental agents, breastfeeding should be discontinued if the mother is treated.
  • Eligibility Criteria Specific to SUB-STUDY A (Brigatinib arm): CLOSED TO ENROLLMENT
  • Participants must meet all eligibility criteria outlined in the Master Study
  • Participants must be willing and able to provide written informed consent/assent for the brigatinib arm of the INTUITT-NF2 trial.
  • Participant is ≥ 12 years of age and has body weight at least 40 kg on Day 1 of treatment.
  • Patient must be able to swallow pills.
  • Clinical laboratory values as specified below within 28 days before the first dose of study drug, as described in the protocol document:
  • Female patients participating in this study should avoid becoming pregnant, and male patients should avoid impregnating a female partner. Non-sterilized female patients of reproductive age group and male patients should use effective methods of contraception through defined periods during and after study treatment as specified below:
  • Female patients must meet 1 of the following:
  • +111 more criteria

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (6)

UCLA Medical Center

Los Angeles, California, 90095, United States

RECRUITING

University of Miami

Miami, Florida, 33136, United States

RECRUITING

Indiana University School of Medicine

Indianopolis, Indiana, 46202, United States

NOT YET RECRUITING

Johns Hopkins Hospital

Baltimore, Maryland, 21287, United States

RECRUITING

Massachusetts General Hospital

Boston, Massachusetts, 02115, United States

RECRUITING

New York University Langone Medical Center

New York, New York, 10016, United States

RECRUITING

Related Publications (1)

  • Plotkin SR, Yohay KH, Nghiemphu PL, Dinh CT, Babovic-Vuksanovic D, Merker VL, Bakker A, Fell G, Trippa L, Blakeley JO; INTUITT-NF2 Consortium. Brigatinib in NF2-Related Schwannomatosis with Progressive Tumors. N Engl J Med. 2024 Jun 27;390(24):2284-2294. doi: 10.1056/NEJMoa2400985. Epub 2024 Jun 21.

MeSH Terms

Conditions

Neurofibromatosis 2Neuroma, AcousticMeningiomaEpendymoma

Interventions

brigatinibneratinibBevacizumab

Condition Hierarchy (Ancestors)

NeurilemmomaNeuroendocrine TumorsNeuroectodermal TumorsNeoplasms, Germ Cell and EmbryonalNeoplasms by Histologic TypeNeoplasmsNeurofibromatosesNeurofibromaNerve Sheath NeoplasmsNeoplasms, Nerve TissueNeuromaNeoplastic Syndromes, HereditaryVestibulocochlear Nerve DiseasesRetrocochlear DiseasesEar DiseasesOtorhinolaryngologic DiseasesOtorhinolaryngologic NeoplasmsCranial Nerve NeoplasmsCranial Nerve DiseasesNervous System DiseasesNeurocutaneous SyndromesHeredodegenerative Disorders, Nervous SystemNeurodegenerative DiseasesGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesNervous System NeoplasmsNeoplasms by SitePeripheral Nervous System NeoplasmsNeoplasms, Vascular TissueMeningeal NeoplasmsCentral Nervous System NeoplasmsGliomaNeoplasms, NeuroepithelialNeoplasms, Glandular and Epithelial

Intervention Hierarchy (Ancestors)

Antibodies, Monoclonal, HumanizedAntibodies, MonoclonalAntibodiesImmunoglobulinsImmunoproteinsBlood ProteinsProteinsAmino Acids, Peptides, and ProteinsSerum GlobulinsGlobulins

Study Officials

  • Scott Plotkin, MD

    Massachusetts General Hospital

    PRINCIPAL INVESTIGATOR

Central Study Contacts

Scott Plotkin, MD

CONTACT

Study Design

Study Type
interventional
Phase
phase 2
Allocation
RANDOMIZED
Masking
NONE
Purpose
TREATMENT
Intervention Model
PARALLEL
Model Details: The study is a platform-basket trial that includes a Master Protocol with embedded Drug Sub-studies. Currently, there are three drug sub-studies: sub-study A (brigatinib), sub-study B (neratinib), and sub-study C (retifanlimab plus bevacizumab). The overall number of arms is not fixed by design because the investigators include arm-dropping rules for futility and allow for the possibility of arm addition by amendment.
Sponsor Type
OTHER
Responsible Party
SPONSOR INVESTIGATOR
PI Title
Sponsor Investigator

Study Record Dates

First Submitted

April 30, 2020

First Posted

May 5, 2020

Study Start

June 20, 2020

Primary Completion (Estimated)

December 1, 2029

Study Completion (Estimated)

December 1, 2030

Last Updated

May 6, 2026

Record last verified: 2026-05

Data Sharing

IPD Sharing
Will share

The Dana-Farber / Harvard Cancer Center encourages and supports the responsible and ethical sharing of data from clinical trials. De-identified participant data from the final research dataset used in the published manuscript may only be shared under the terms of a Data Use Agreement. Requests may be directed to Sponsor Investigator or designee. The protocol and statistical analysis plan will be made available on Clinicaltrials.gov only as required by federal regulation or as a condition of awards and agreements supporting the research.

Shared Documents
STUDY PROTOCOL, SAP, ICF
Time Frame
Data can be shared no earlier than 1 year following the date of publication
Access Criteria
MGH - Contact the Partners Innovations team at http://www.partners.org/innovation

Locations