Clinical Trial

PNOC013: A Safety and Pharmacokinetic Study of Single Agent REGN2810 in Pediatric Patients with Relapsed or Refractory Solid or Central Nervous System (CNS) Tumors and a Safety and Efficacy Trial of REGN2810 in Combination with Radiotherapy in Pediatric Patients

Please note, this study has closed to accrual and is no longer accepting patients. 

Patients with solid tumors will receive REGN2810 intravenously (IV) every 2 weeks. This phase of the study will confirm the pediatric dose of REGN2810 and will not include treatment with radiation. DIPG and HGG patients will receive REGN2810 via IV every 2 weeks in combination with radiotherapy and then as monotherapy once radiotherapy is complete. Newly diagnosed patients with DIPG or HGG will be randomized to receive either standard or hypofractionated radiation; patients with recurrent HGG will receive standard re-irradiation.

The median survival for newly diagnosed DIPG and recurrent HGG is less than 1 year, while only 20% of patients with newly diagnosed HGG are alive at 2 years. Immunotherapy is a novel therapeutic approach that is emerging as effective therapy for a variety of malignancies and may have significant potential in the treatment of pediatric DIPG and HGG as well as other solid tumors. Preclinical data have shown that radiation “primes” the immune system as evidenced by increased antigen presentation, increased major histocompatibility complex (MHC) expression, and increased antigen-specific T-cells in the tumor microenvironment. Given these findings, combining radiation with PD-1 inhibition in children with DIPG and HGGs makes biological and clinical sense.

PRIMARY OBJECTIVES

To determine the safety and recommended phase 2 dose of REGN2810 for children with recurrent solid or CNS tumors, and newly diagnosed DIPG or HGG and recurrent HGG when given with radiotherapy.

SECONDARY OBJECTIVES

To assess safety and tolerability of REGN2810 when given in combination with radiotherapy, and to assess immunogenicity.

INCLUSION CRITERIA

Key Inclusion Criteria:

  • Age 0 to <18 years of age (Phase 1)
  • Age ≥3 and ≤25 years of age (Efficacy Phase)
  • Karnofsky ≥50 for patients >16 years of age and Lansky ≥50 for patients ≤ 16 years of age
  • Patients who are receiving corticosteroids must be on a stable or decreasing dose for at least 7 days prior to enrollment
  • Adequate Bone Marrow Function
  • Adequate Renal Function
  • Adequate Liver Function
  • Adequate Neurologic Function

 EXCLUSION CRITERIA

Key Exclusion Criteria:

  • Patients with bulky, metastatic disease of the CNS causing uncal herniation or symptomatic midline shift; significant, symptomatic mass effect; or uncontrolled neurological symptoms such as seizures or altered mental status
  • Patients with metastatic spine disease and gliomatosis as documented by diffuse involvement of greater than 2 lobes
  • Patients who are receiving any other investigational agents
  • Patients on greater than dexamethasone 0.1 mg/kg/day (maximum 4 mg/day) or equivalent dose in alternate corticosteroid or actively undergoing corticosteroid dose escalation
  • Patients with a history of allogeneic stem cell transplant
  • Prior treatment with an agent that blocks the PD-1/PD-L1/PD-L2 pathway

Note: Other protocol Inclusion/Exclusion criteria apply

Please note, this study has closed to accrual and is no longer accepting patients. 

Patients with solid tumors will receive REGN2810 intravenously (IV) every 2 weeks. This phase of the study will confirm the pediatric dose of REGN2810 and will not include treatment with radiation. DIPG and HGG patients will receive REGN2810 via IV every 2 weeks in combination with radiotherapy and then as monotherapy once radiotherapy is complete. Newly diagnosed patients with DIPG or HGG will be randomized to receive either standard or hypofractionated radiation; patients with recurrent HGG will receive standard re-irradiation.

The median survival for newly diagnosed DIPG and recurrent HGG is less than 1 year, while only 20% of patients with newly diagnosed HGG are alive at 2 years. Immunotherapy is a novel therapeutic approach that is emerging as effective therapy for a variety of malignancies and may have significant potential in the treatment of pediatric DIPG and HGG as well as other solid tumors. Preclinical data have shown that radiation “primes” the immune system as evidenced by increased antigen presentation, increased major histocompatibility complex (MHC) expression, and increased antigen-specific T-cells in the tumor microenvironment. Given these findings, combining radiation with PD-1 inhibition in children with DIPG and HGGs makes biological and clinical sense.

PRIMARY OBJECTIVES

To determine the safety and recommended phase 2 dose of REGN2810 for children with recurrent solid or CNS tumors, and newly diagnosed DIPG or HGG and recurrent HGG when given with radiotherapy.

SECONDARY OBJECTIVES

To assess safety and tolerability of REGN2810 when given in combination with radiotherapy, and to assess immunogenicity.

INCLUSION CRITERIA

Key Inclusion Criteria:

  • Age 0 to <18 years of age (Phase 1)
  • Age ≥3 and ≤25 years of age (Efficacy Phase)
  • Karnofsky ≥50 for patients >16 years of age and Lansky ≥50 for patients ≤ 16 years of age
  • Patients who are receiving corticosteroids must be on a stable or decreasing dose for at least 7 days prior to enrollment
  • Adequate Bone Marrow Function
  • Adequate Renal Function
  • Adequate Liver Function
  • Adequate Neurologic Function

 EXCLUSION CRITERIA

Key Exclusion Criteria:

  • Patients with bulky, metastatic disease of the CNS causing uncal herniation or symptomatic midline shift; significant, symptomatic mass effect; or uncontrolled neurological symptoms such as seizures or altered mental status
  • Patients with metastatic spine disease and gliomatosis as documented by diffuse involvement of greater than 2 lobes
  • Patients who are receiving any other investigational agents
  • Patients on greater than dexamethasone 0.1 mg/kg/day (maximum 4 mg/day) or equivalent dose in alternate corticosteroid or actively undergoing corticosteroid dose escalation
  • Patients with a history of allogeneic stem cell transplant
  • Prior treatment with an agent that blocks the PD-1/PD-L1/PD-L2 pathway

Note: Other protocol Inclusion/Exclusion criteria apply

How to Enroll

If you believe your child or patient is eligible for this trial, contact the closest participating site or email us for more information.