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Study Of Entrectinib (Rxdx-101) in Children and Adolescents With Locally Advanced Or Metastatic Solid Or Primary CNS Tumors And/Or Who Have No Satisfactory Treatment Options

Phase 1
Active, not recruiting
Conditions
Solid Tumors
CNS Tumors
Interventions
Registration Number
NCT02650401
Lead Sponsor
Hoffmann-La Roche
Brief Summary

This is an open-label, Phase 1/2 multicenter dose escalation study in pediatric patients with relapsed or refractory extracranial solid tumors (Phase 1), with additional expansion cohorts (Phase 2) in patients with primary brain tumors harboring NTRK1/2/3 or ROS1 gene fusions, and extracranial solid tumors harboring NTRK1/2/3 or ROS1 gene fusions.

Detailed Description

Not available

Recruitment & Eligibility

Status
ACTIVE_NOT_RECRUITING
Sex
All
Target Recruitment
69
Inclusion Criteria
  1. Disease status:

    • Phase 1 portion (closed): Participants must have measurable or evaluable disease, as defined by RECIST v1.1

    • Phase 2 portion:

      • Part B: Participants must have measurable or evaluable disease, as defined by RANO
      • Part C (closed): Participants must have measurable or evaluable disease, as defined by RECIST v1.1 ± Curie Scale
      • Part D: Participants must have measurable or evaluable disease, as defined by RECIST v1.1
      • Part E (closed): Participants must have measurable or evaluable disease, as defined by RECIST v1.1 ± Curie Scale or RANO
  2. Tumor type:

    • Phase 1 portion:

      * Part A: Relapsed or refractory extracranial solid tumors

    • Phase 2 portion

      • Part B: Primary brain tumors with NTRK1/2/3 or ROS1 gene fusions; gene fusions are defined as those predicted to translate into a fusion protein with a functional TRKA/B/C or ROS1 kinase domain, without a concomitant second oncodriver as determined by a nucleic acid-based diagnostic testing method
      • Part D: Extracranial solid tumors (including NB) with NTRK1/2/3 or ROS1 gene fusions; gene fusions are defined as those predicted to translate into a fusion protein with a functional TRKA/B/C or ROS1 kinase domain, without a concomitant second oncodriver as determined by a nucleic acid-based diagnostic testing method
  3. Histologic/molecular diagnosis of malignancy at diagnosis or the time of relapse

  4. Archival tumor tissue from diagnosis or, preferably, at relapse

  5. Performance status: Lansky or Karnofsky score ≥ 60% and minimum life expectancy of at least 4 weeks

  6. Prior therapy: Participants must have a disease that is locally advanced, metastatic, or where surgical resection is likely to result in severe morbidity, and who have no satisfactory treatment options for solid tumors and primary CNS tumors that are neurotrophic tyrosine receptor kinase (NTRK) or ROS1 fusion-positive

  7. Participants must have recovered from the acute toxic effects of all prior chemotherapy, immunotherapy, or radiotherapy prior to enrollment

  8. Adequate organ and neurologic function

  9. Females of childbearing potential must have a negative serum pregnancy test during screening and be neither breastfeeding nor intending to become pregnant during study participation. Agreement to remain abstinent or use use combined contraceptive methods prior to study entry, for the duration of study participation and in the following 90 days after discontinuation of study treatment.

  10. For male participants with a female partner of childbearing potential or a pregnant female partner: Agreement to remain abstinent or use a condom during the treatment period and for at least 3 months after the last dose of study drug

Exclusion Criteria
  1. Receiving other experimental therapy
  2. Known congenital long QT syndrome
  3. History of recent (3 months) symptomatic congestive heart failure or ejection fraction ≤50% at screening
  4. Known active infections
  5. Familial or personal history of congenital bone disorders, bone metabolism alterations or osteopenia
  6. Receiving Enzyme Inducing Antiepileptic Drugs (EIAEDs) within 14 days of first dose.
  7. Prior treatment with approved or investigational TRK or ROS1 inhibitors
  8. Known hypersensitivity to entrectinib or any of the other excipients of the investigational medicinal product
  9. Patients with NB with bone marrow space-only disease
  10. Incomplete recovery from acute effects of any surgery prior to treatment.
  11. Active gastrointestinal disease or other malabsorption syndromes that would impact drug absorption.
  12. Other severe acute or chronic medical or psychiatric condition or lab abnormality that may increase the risk associated with study participation, drug administration or may interfere with the interpretation of study results.

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Expansion: CNS tumors harboring NTRK1/2/3, ROS1Entrectinibgene fusions Oral entrectinib (RXDX-101)
Any participant unable to swallow capsulesEntrectinibArm closed for further enrollment Any participant who otherwise meet all other eligibility criteria Oral entrectinib (RXDX-101)
Extracranial solid tumors harboring NTRK1/2/3,EntrectinibArm closed for further enrollment ROS1, ALK non-gene fusion molecular alterations Oral entrectinib (RXDX-101)
CNS tumors harboring- NTRK1/2/3, ROS1, ALKEntrectinibArm closed for further enrollment molecular alterations, including gene fusions Oral entrectinib (RXDX-101)
Non-neuroblastoma, extracranial solid tumorsEntrectinibArm closed for further enrollment harboring - NTRK1/2/3, ROS1, ALK gene fusions Oral entrectinib (RXDX-101)
Expansion: Extracranial solid tumors harboring NTRK1/2/3, ROS1EntrectinibNTRK 1,2,3 and ROS1 fusions Oral entrectinib (RXDX-101)
NeuroblastomaEntrectinibArm closed for further enrollment Oral entrectinib (RXDX-101)
Primary Outcome Measures
NameTimeMethod
Maximum Tolerated Dose (MTD)Approximately 6 months

Assessed by National Cancer Institute Common Terminology for Adverse Events Criteria (NCI CTCAE v4.03)

Recommended Phase 2 Dose (RP2D) Of Minitablets/F15 Formulation In Pediatric Participants Unable To Swallow Intact CapsulesApproximately 6 months

Assessed by NCI CTCAE v4.03

Cohort B: Objective Response Rate (ORR)Approximately 6 months

Assessed by RANO per the BICR

Recommended Phase 2 Dose (RP2D) of F06 Formulation In Pediatric Participants Able To Swallow Intact CapsulesApproximately 6 months

Assessed by NCI CTCAE v4.03

Recommended Phase 2 Dose (RP2D) of F1 Formulation In Pediatric Participants Able To Swallow Intact CapsulesApproximately 6 months

Assessed by NCI CTCAE v4.03

Recommended Phase 2 Dose (RP2D) of F06 Formulation In Pediatric In Participants Dosed Via Feeding Tube (Nasogastric Tube Or Gastric Tube)Approximately 6 months

Assessed by NCI CTCAE v4.03

Cohort D: ORRApproximately 6 months

Assessed by RECIST v1.1 per the BICR

Secondary Outcome Measures
NameTimeMethod
Cohort B or E: OSApproximately 6 months

Assessed by RANO

Maximum observed plasma drug concentration (Cmax) using F1 FormulationApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

AUC at steady state (AUCss) using F1 FormulationApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Safety and Tolerability - AE, ECG and Labs assessed by NCI CTCAE v4.03Approximately 24 months

AE, ECG and Labs assessed by NCI CTCAE v4.03

Maximum observed plasma drug concentration (Cmax) using minitablets/F15Approximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Cohort B or E: DORApproximately 6 months

Assessed by RANO per the BICR and investigator

Cohort C: DORApproximately 6 months

Assessed by the Curie scale per the BICR and investigator

Maximum observed plasma drug concentration (Cmax) using F06 Formulation given intactApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Maximum observed plasma drug concentration (Cmax) using F06 Formulation administered via feeding tubeApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Time to Cmax, by inspection (Tmax) using F1 FormulationApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Time to Cmax, by inspection (Tmax) using F06 Formulation given intactApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Terminal half life (t½) using F06 Formulation given intactApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Terminal half life (t½) using F06 Formulation administered via feeding tubeApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Cohort A, D, or E: Clinical Benefit Rate (CBR)Approximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Cohort B or E: PFSApproximately 6 months

Assessed by RANO per the BICR and investigator

Phase 1/2 Participants with NTRK1/2/3 gene fusions (Cohort B or E): ORRApproximately 6 months

Assessed by RANO per the BICR and investigator

Phase 1/2 Participants with NTRK1/2/3 or ROS1 gene fusions (Cohort A, D, or E): ORRApproximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Phase 1/2 Participants with NTRK1/2/3 or ROS1 gene fusions (Cohort B or E): ORRApproximately 6 months

Assessed by RANO per the BICR and investigator

Phase 2 Participants with NTRK1/2/3 or ROS1 gene fusions (Cohort B or E): DORApproximately 6 months

Assessed by RANO per the BICR and investigator

Phase 1/2 Participants with ROS1 gene fusions (Cohort A, D, or E): DORApproximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Phase 1/2 Participants with NTRK1/2/3 or ROS1 gene fusions (Cohort A, D, or E): DORApproximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Cohort B or E: TTRApproximately 6 months

Assessed by RANO per the BICR and investigator

Time to Cmax, by inspection (Tmax) using F06 Formulation administered via feeding tubeApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Time to Cmax, by inspection (Tmax) using minitablets/F15Approximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

AUC at steady state (AUCss) using F06 Formulation administered via feeding tubeApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Terminal half life (t½) using F1 FormulationApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Terminal half life (t½) using minitablets/F15Approximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Area under the drug concentration by time curve (AUC) using F1 FormulationApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Area under the drug concentration by time curve (AUC) using F06 Formulation administered via feeding tubeApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Cohort C: CBRApproximately 6 months

Assessed by the Curie scale per the BICR and investigator

Cohort A, D, or E: ORRApproximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Cohort A, D, or E: Time to response (TTR)Approximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Cohort C: TTRApproximately 6 months

Assessed by the Curie scale per the BICR and investigator

Phase 2 Participants with NTRK1/2/3 or ROS1 gene fusions (Cohort B or E): ORRApproximately 6 months

Assessed by RANO per the investigator

Phase 2 Participants with NTRK1/2/3 or ROS1 gene fusions (Cohort D or E): ORRApproximately 6 months

Assessed by RECIST v1.1 per the investigator

Phase 1/2 Participants with NTRK1/2/3 gene fusions (Cohort A, D, or E): ORRApproximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

AUC at steady state (AUCss) using minitablets/F15Approximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Area under the drug concentration by time curve (AUC) using minitablets/F15Approximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Cohort A, D, or E: Progression-free Survival (PFS)Approximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Cohort C: PFSApproximately 6 months

Assessed by the Curie scale per the BICR and investigator

Cohort A, D, or E: Overall Survival (OS)Approximately 6 months

Assessed by RECIST v1.1

Cohort C: ORRApproximately 6 months

Assessed by the Curie scale per the BICR and investigator

AUC at steady state (AUCss) using F06 Formulation given intactApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Cohort B or E: ORRApproximately 6 months

Assessed by RANO per the BICR and investigator

Phase 1/2 Participants with ROS1 gene fusions (Cohort B or E): ORRApproximately 6 months

Assessed by RANO per the BICR and investigator

Phase 1/2 Participants with ROS1 gene fusions (Cohort B or E): TTRApproximately 6 months

Assessed by RANO per the BICR and investigator

Area under the drug concentration by time curve (AUC) using F06 Formulation given intactApproximately 24 months

Assessed by plasma concentrations obtained on Days 1, 2, 8, 15, 22 (Cycle 1), Days 1, 2 (Cycle 2) and on Day 1 of every cycle thereafter

Cohort B or E: CBRApproximately 6 months

Assessed by RANO per the BICR and investigator

Cohort A, D, or E: Duration of Response (DOR)Approximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Phase 1/2 Participants with ROS1 gene fusions (Cohort A, D, or E): ORRApproximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Phase 1/2 Participants with ROS1 gene fusions (Cohort B or E): DORApproximately 6 months

Assessed by RANO per the BICR and investigator

Phase 2 Participants with NTRK1/2/3 or ROS1 gene fusions (Cohort D or E): DORApproximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Phase 1/2 Participants with NTRK1/2/3 gene fusions (Cohort A, D, or E): DORApproximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Phase 1/2 Participants with NTRK1/2/3 gene fusions (Cohort B or E): DORApproximately 6 months

Assessed by RANO per the BICR and investigator

Phase 1/2 Participants with NTRK1/2/3 or ROS1 gene fusions (Cohort B or E): DORApproximately 6 months

Assessed by RANO per the BICR and investigator

Phase 1/2 Participants with NTRK1/2/3 gene fusions (Cohort A, D, or E): TTRApproximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Phase 2 Participants with NTRK1/2/3 or ROS1 gene fusions (Cohort B or E): TTRApproximately 6 months

Assessed by RANO per the BICR and investigator

Phase 2 Participants with NTRK1/2/3 or ROS1 gene fusions (Cohort D or E): TTRApproximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Phase 1/2 Participants with ROS1 gene fusions (Cohort A, D, or E): TTRApproximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Phase 1/2 Participants with NTRK1/2/3 gene fusions (Cohort B or E): TTRApproximately 6 months

Assessed by RANO per the BICR and investigator

Phase 1/2 Participants with NTRK1/2/3 or ROS1 gene fusions (Cohort A, D, or E): TTRApproximately 6 months

Assessed by RECIST v1.1 per the BICR and investigator

Phase 1/2 Participants with NTRK1/2/3 or ROS1 gene fusions (Cohort B or E): TTRApproximately 6 months

Assessed by RANO per the BICR and investigator

Trial Locations

Locations (26)

Texas Children's Cancer and Hematology Center

🇺🇸

Houston, Texas, United States

UCSF Benioff Children's Hospital

🇺🇸

San Francisco, California, United States

Children's Hospital Colorado

🇺🇸

Aurora, Colorado, United States

Egleston Children's Hospital at Emory University Atlanta

🇺🇸

Atlanta, Georgia, United States

University of Chicago

🇺🇸

Chicago, Illinois, United States

Washington University,St. Louis Children's Hospital

🇺🇸

Saint Louis, Missouri, United States

Memorial Sloan Kettering Cancer Center

🇺🇸

New York, New York, United States

Nationwide Children's Hospital

🇺🇸

Columbus, Ohio, United States

Beijing Children's Hospital, Capital Medical University

🇨🇳

Beijing, China

Centre Leon Berard

🇫🇷

Lyon, France

Fondazione IRCCS Istituto Nazionale dei Tumori

🇮🇹

Milano, Lombardia, Italy

Royal Victoria Infirmary

🇬🇧

Newcastle upon Tyne, United Kingdom

Rady Childrens Hospital

🇺🇸

San Diego, California, United States

Johns Hopkins University

🇺🇸

Baltimore, Maryland, United States

Dana Farber Cancer Institute

🇺🇸

Boston, Massachusetts, United States

Oregon Health & Science Uni

🇺🇸

Portland, Oregon, United States

Children's Hospital of Philadelphia

🇺🇸

Philadelphia, Pennsylvania, United States

St. Jude Children'S Research Hospital

🇺🇸

Memphis, Tennessee, United States

Primary Children's Hospital

🇺🇸

Salt Lake City, Utah, United States

The Hospital for Sick Children

🇨🇦

Toronto, Ontario, Canada

Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine

🇨🇳

Shanghai, China

Hôpital de la Timone, Oncologie Pédiatrique

🇫🇷

Marseille, France

Universitaetsklinikum Heidelberg

🇩🇪

Heidelberg, Baden Wuerttemberg, Germany

Hong Kong Children's Hospital

🇭🇰

Hong Kong, Hong Kong

Hospital Infantil Universitario Nino Jesus

🇪🇸

Madrid, Spain

Royal Marsden NHS Foundation Trust

🇬🇧

Sutton, United Kingdom

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