Tiragolumab and Atezolizumab for the Treatment of Relapsed or Refractory SMARCB1 or SMARCA4 Deficient Tumors

Conditions

• Atypical Teratoid/Rhabdoid Tumor
• Epithelioid Sarcoma
• Kidney Medullary Carcinoma
• Malignant Solid Neoplasm
• Poorly Differentiated Chordoma
• Recurrent Atypical Teratoid/Rhabdoid Tumor
• Recurrent Chordoma
• Recurrent Epithelioid Sarcoma
• Recurrent Kidney Medullary Carcinoma
• Recurrent Malignant Solid Neoplasm
• Recurrent Rhabdoid Tumor
• Refractory Atypical Teratoid/Rhabdoid Tumor
• Refractory Chordoma
• Refractory Epithelioid Sarcoma
• Refractory Kidney Medullary Carcinoma
• Refractory Malignant Solid Neoplasm
• Refractory Rhabdoid Tumor
• Rhabdoid Tumor

Eligibility Criteria

Sex

ALL

Age

12 Months - N/A

Healthy Volunteers

Not accepted

Interventions

• Atezolizumab — BIOLOGICAL
  Given IV
• Biospecimen Collection — PROCEDURE
  Undergo blood sample collection
• Computed Tomography — PROCEDURE
  Undergo CT and/or PET-CT
• Echocardiography Test — PROCEDURE
  Undergo ECHO
• Fludeoxyglucose F-18 — OTHER
  Given FDG
• Magnetic Resonance Imaging — PROCEDURE
  Undergo MRI
• Positron Emission Tomography — PROCEDURE
  Undergo PET-CT and/or FDG-PET
• Tiragolumab — BIOLOGICAL
  Given IV
• X-Ray Imaging — PROCEDURE
  Undergo x-rays

Study Details

This phase I/II trial studies how well tiragolumab and atezolizumab works when given to children and adults with SMARCB1 or SMARCA4 deficient tumors that have either come back (relapsed) or do not respond to therapy (refractory). SMARCB1 or SMARCA4 deficiency means that tumor cells are missing the SMARCB1 and SMARCA4 genes, seen with some aggressive cancers that are typically hard to treat. Immunotherapy with monoclonal antibodies, such as tiragolumab and atezolizumab, may help the body's immune system attack the cancer, and may interfere with the ability of tumor cells to grow and spread.

Key Dates

Start date

Nov 17, 2022

Status verified

Jan 2026

Primary completion

Mar 31, 2030

Completion

Mar 31, 2030

Study Design

Enrollment

86 participants (estimated)

Allocation

NON_RANDOMIZED

Intervention model

PARALLEL

Primary purpose

TREATMENT

Arms

• Experimental: Arm B (atezolizumab, tiragolumab)
  Patients receive atezolizumab IV over 30-60 minutes on day 1 and tiragolumab IV over 30-90 minutes on day 1 of each cycle. Treatment repeats every 21 days for up to 5 years in the absence of disease progression or unacceptable toxicity. Patients also undergo standard imaging scans including x-rays, CT, MRI, and/or FDG PET-CT throughout the trial. Patients also undergo ECHO during screening and blood sample collection on study.
• Experimental: Part A (atezolizumab, tiragolumab)
  Patients receive tiragolumab IV over 30-90 minutes on day 1 of each cycle and atezolizumab IV over 30-60 minutes on day 1 of each cycle starting in cycle 2. Treatment repeats every 21 days for up to 5 years in the absence of disease progression or unacceptable toxicity. Patients undergo standard imaging scans including x-rays, CT, MRI, and/or FDG PET-CT, throughout the trial. Patients also undergo ECHO during screening and blood sample collection on study.

Primary Outcome Measure

Frequency of cycle 1 dose limiting toxicities of tiragolumab as monotherapy in pediatric patients [ Time Frame: Up to 21 days ]

Locations (35)

Find similar trials in Birmingham, AL

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