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A Study to Evaluate Tovorafenib in Pediatric and Young Adult Participants With Relapsed or Progressive Low-Grade Glioma and Advance Solid Tumors

Phase 2
Recruiting
Conditions
Low-grade Glioma
Advanced Solid Tumor
Interventions
Registration Number
NCT04775485
Lead Sponsor
Day One Biopharmaceuticals, Inc.
Brief Summary

This is a Phase 2, multi center, open-label study to evaluate the safety and efficacy of Type II RAF (tovorafenib) in pediatric participants with low-grade glioma or advanced solid tumors. Qualifying genomic alterations will be identified through molecular assays as routinely performed at Clinical Laboratory Improvement Amendments (CLIA) of 1988 or other similarly certified laboratories prior to enrollment into any of the arms. The study will consist of a screening period, a treatment period, a long-term extension phase, end of treatment (EOT) visit(s), a safety follow-up visit, and long-term follow-up assessments.

Detailed Description

The study will consist of the following treatment arms:

Arm 1 (Low-Grade Glioma): Patients aged 6 months to 25 years, inclusive, with recurrent or progressive low-grade glioma harboring a known activating BRAF alteration, including BRAF V600 mutations and KIAA1549:BRAF fusions.

Arm 2 (Low-Grade Glioma Expanded Access): Patients aged 6 months to 25 years, inclusive, with recurrent or progressive low-grade glioma harboring a known or expected to be activating RAF alteration (e.g., BRAF or CRAF/RAF1 fusion or BRAF V600 mutations). Opening of Arm 2 to enrollment will be based on the recommendation of the Data Safety Monitoring Board (DSMB).

Arm 3 (Advanced Solid Tumor): Patients aged 6 months to 25 years, inclusive, with advanced solid tumors harboring a known or expected to be activating RAF fusion (e.g., BRAF or CRAF/RAF1 fusion).

Qualifying genomic alterations will be identified through molecular assays as routinely performed at Clinical Laboratory Improvement Amendments (CLIA) of 1988 or other similarly certified laboratories prior to enrollment into any of the aforementioned arms.

Patients will be treated with DAY101, an oral pan-RAF inhibitor, for a planned period of 26 cycles will be treated with DAY101 for a planned period of 26 cycles (approximately 24 months).

DAY101 will be administered at the recommended Phase 2 dose (RP2D) of 420 mg/m2 (not to exceed 600 mg) orally once weekly (QW) for each 28-day treatment cycle.

Treatment cycles will repeat every 28 days in the absence of disease progression or unacceptable toxicity. Patients will undergo radiographic evaluation of their disease at the end of every third cycle. Patients will continue on DAY101 until radiographic evidence of disease progression by RANO (Arms 1 \& 2) or RECIST v1.1 criteria (Arm 3) as determined by treating investigator, unacceptable toxicity, patient withdrawal of consent, or death.

Patients who have radiographic evidence of disease progression may be allowed to continue DAY101 if, in the opinion of the investigator and approval by the Sponsor, the patient is deriving clinical benefit from continuing study treatment. Disease assessments for patients being treated beyond progression should continue as per regular schedule.

DAY101 is an oral pan-RAF inhibitor administered as an oral tablet at 420 mg/m2 (not to exceed 600 mg).

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
141
Inclusion Criteria
  • Low Grade Glioma & Low-Grade Glioma Extension: a relapsed or progressive LGG with documented known activating BRAF alteration.
  • Advanced Solid Tumor: locally advanced or metastatic solid tumor with documented known or expected to be activating RAF fusion.
  • Participants must have histopathologic verification of malignancy at either original diagnosis or relapse.
  • Must have received at least one line of prior systemic therapy and have documented evidence of radiographic progression.
  • Must have at least 1 measurable lesion as defined by RANO (Arms 1 & 2) or RECIST v1.1 (Arm 3) criteria
Exclusion Criteria
  • Participant's tumor has additional previously-known activating molecular alterations.
  • Participant has symptoms of without radiographically recurrent or radiographically progressive disease.
  • Known or suspected diagnosis of neurofibromatosis type 1 (NF-1) via genetic testing or current diagnostic criteria.

Other inclusion/exclusion criteria as stipulated by protocol may apply

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Arm 1: Low-Grade GliomaTovorafenibParticipants with recurrent or progressive low-grade glioma will receive 420 milligrams/meters square (mg/m\^2) of tovorafenib weekly according to dose rounding guidelines and according to their baseline body surface area (BSA).
Arm 2: Low-Grade Glioma Expanded AccessTovorafenibParticipants with recurrent or progressive low-grade glioma will receive 420 mg/m\^2 of tovorafenib weekly according to dose rounding guidelines and according to their baseline BSA.
Arm 3: Advanced Solid TumorTovorafenibParticipants with advanced solid tumors will receive 420 mg/m\^2) of tovorafenib weekly according to dose rounding guidelines and according to their baseline BSA.
Primary Outcome Measures
NameTimeMethod
Arm 1: Overall response rateUp to 48 months

ORR is defined as percentage of participants with best overall confirmed response of complete response (CR) or partial response (PR) by the Response Assessment in Neuro-Oncology - high-grade glioma (RANO-HGG) criteria.

Arm 2: Number of participants reporting adverse eventsUp to 48 months

An adverse event (AE) is any untoward medical occurrence in a participant or clinical study participant, temporally associated with the use of study intervention, whether or not considered related to the study intervention.

Arm 2: Number of participants with clinically significant changes in clinical chemistry parametersUp to 48 months
Arm 2: Number of participants with clinically significant changes in hematology parametersUp to 48 months
Arm 3: Overall response rateUp to 48 months

Determined by the treating investigator and measured by Response Evaluation Criteria in Solid Tumors (RECIST) v1.1 or RANO-HGG criteria, as appropriate.

Secondary Outcome Measures
NameTimeMethod
Arm 1 and 3: Number of participants with clinically significant changes in hematology parametersUp to 48 months
Arm 1: Area under the concentration-time curve (AUC) of TovorafenibCycle 1: Day 1 and Day 15; Cycles 2, 4, 7, 10 and 13: Day 1
Arm 1: Minimum drug concentration (Cmin)Cycle 1: Day 1 and Day 15; Cycles 2, 4, 7, 10 and 13: Day 1
Arm 1: Change from Baseline QT interval corrected for heart rate by Fridericia's formula (ΔQTcF)Baseline to 48 months
Arm 1: Change from Baseline PR interval (ΔPR)Baseline to 48 months
Arm 1: Change from Baseline QRS interval (ΔQRS)Baseline to 48 months
Arm 1: Change from baseline heart rate (ΔHR)Baseline to 48 months
Arm 1: Change in electrocardiogram (ECG) waveform morphologyBaseline to 48 months
Arm 1 and Arm 2: Overall response rateUp to 48 months

ORR is defined as percentage of participants with best overall confirmed response of CR or PR by the RANO-HGG criteria.

Arm 1 and 3: Number of participants reporting adverse eventsUp to 48 months

An adverse event (AE) is any untoward medical occurrence in a participant or clinical study participant, temporally associated with the use of study intervention, whether or not considered related to the study intervention.

Arm 1 and 3: Number of participants with clinically significant changes in clinical chemistry parametersUp to 48 months
Arm 1, Arm 2 and Arm 3: Overall response rate in Pediatric participantsUp to 48 months

ORR is defined as percentage of participants with best overall confirmed response of CR or PR or minor response (MR) by Response Assessment in Pediatric Neuro-Oncology (RAPNO) criteria.CR or PR by RECIST v1.1 criteria.

Arm 1, Arm 2 and Arm 3: duration of progression-free survival (PFS)Up to 48 months

PFS as defined by the time following initiation of tovorafenib to progression or death in participants treated with tovorafenib measured by RECIST v1.1, RAPNO, or RANO-HGG criteria as determined by the treating investigator and an IRC.

Arm 1, Arm 2 and Arm 3: Duration of response (DOR)Up to 48 months

DOR as defined by the length of response in participants with best overall confirmed response of CR or PR or MR and measured by RANO-HGG, RAPNO, and/or RECIST v1.1 criteria, as applicable.

Arm 1, Arm 2 and Arm 3: Time to response (TTR)Up to 48 months

TTR as defined as the time to first response following initiation of tovorafenib in participants with best overall confirmed response of CR or PR measured by RECIST v1.1 or RANO-HGG criteria, as applicable.

Arm 1, Arm 2 and Arm 3: Clinical benefit rate (CBR)Up to 48 months

CBR as defined as participants with BOR of CR, PR or stable disease (SD) measured by RECIST v1.1 or RANO-HGG, as applicable, and lasting 12 months or more following initiation of tovorafenib.

Arm 1 and Arm 2: Duration of overall survivalUp to 48 months

Overall survival as defined by the time following initiation of tovorafenib to death of any cause in participants treated with tovorafenib.

Arm 1: Change from baseline in best corrected visual acuity (BCVA) outcomesBaseline to 48 months
Arm 1: Changes in molecular analysis of cells obtained from archival tissueAt Screening

Trial Locations

Locations (35)

Montreal Children's Hospital

🇨🇦

Montreal, Quebec, Canada

University of Utah

🇺🇸

Salt Lake City, Utah, United States

Sydney Children's Hospital

🇦🇺

Randwick, Australia

Rambam Health Care Campus

🇮🇱

Haifa, Israel

Royal Children's Hospital

🇦🇺

Parkville, Australia

Severance Hospital - Yonsei University

🇰🇷

Seoul, Korea, Republic of

Lurie Children's Hospital of Chicago

🇺🇸

Chicago, Illinois, United States

St. Louis Children's Hospital

🇺🇸

Saint Louis, Missouri, United States

Duke Cancer Center

🇺🇸

Durham, North Carolina, United States

Texas Children's Hospital

🇺🇸

Houston, Texas, United States

Charité Universitätsmedizin Berlin, Campus Virchow Klinikum, Otto-Heubner-Centrum für Kinder

🇩🇪

Berlin, Germany

The Children's Hospital at Westmead

🇦🇺

Westmead, Australia

UCL Great Ormond Street Institute of Child Health

🇬🇧

London, United Kingdom

Hopp-Kindertumorzentrum Heidelberg (KiTZ), KiTZ Clinical Trial Unit (ZIPO)

🇩🇪

Heidelberg, Germany

Children's National Medical Center

🇺🇸

Washington, District of Columbia, United States

Schneider Children's Medical Center of Israel

🇮🇱

Petah Tikva, Israel

Perth Children's Hospital

🇦🇺

Perth, Australia

Centre Mère-Enfant Soleil du CHU

🇨🇦

Québec, Quebec, Canada

CS Mott Children's Hospital

🇺🇸

Ann Arbor, Michigan, United States

Centre Hospitalier Universitaire Ste-Justine

🇨🇦

Montreal, Quebec, Canada

NYU Langone Health

🇺🇸

New York, New York, United States

Queensland Children's Hospital

🇦🇺

Brisbane, Australia

Seoul National University Hospital

🇰🇷

Seoul, Korea, Republic of

UCSF Benioff Children's Hospital

🇺🇸

San Francisco, California, United States

Princess Maxima Center for Pediatric Oncology

🇳🇱

Utrecht, Netherlands

The Chaim Sheba Medical Center

🇮🇱

Ramat Gan, Israel

Children's Hospital of Philadelphia

🇺🇸

Philadelphia, Pennsylvania, United States

Dana-Farber Cancer Institute

🇺🇸

Boston, Massachusetts, United States

Johns Hopkins Hospital

🇺🇸

Baltimore, Maryland, United States

KK Women's and Children's Hospital

🇸🇬

Singapore, Singapore

Universitäts-Kinderspital Zürich - Eleonorenstiftung

🇨🇭

Zürich, Switzerland

Newcastle University

🇬🇧

Newcastle Upon Tyne, United Kingdom

Rigshospitalet

🇩🇰

Copenhagen, Denmark

Doernbecher Children's Hospital Oregon & Health Science University

🇺🇸

Portland, Oregon, United States

Seattle Children's Hospital

🇺🇸

Seattle, Washington, United States

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