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

Summary

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.

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

Interventions

BIOLOGICAL

Atezolizumab

Given IV

PROCEDURE

Biospecimen Collection

Undergo blood sample collection

PROCEDURE

Computed Tomography

Undergo CT and/or PET-CT

PROCEDURE

Echocardiography Test

Undergo ECHO

OTHER

Fludeoxyglucose F-18

Given FDG

PROCEDURE

Magnetic Resonance Imaging

Undergo MRI

PROCEDURE

Positron Emission Tomography

Undergo PET-CT and/or FDG-PET

BIOLOGICAL

Tiragolumab

Given IV

PROCEDURE

X-Ray Imaging

Undergo x-rays

Sponsors & Collaborators

• National Cancer Institute (NCI)

  lead NIH

Principal Investigators

• Mary F Wedekind Malone · Pediatric Early Phase Clinical Trial Network

Study Design

Allocation

NON_RANDOMIZED

Purpose

TREATMENT

Masking

NONE

Model

PARALLEL

Eligibility

Min Age

12 Months

Sex

ALL

Healthy Volunteers

No

Timeline & Regulatory

Start

2022-11-17

Primary Completion

2030-03-31

Completion

2030-03-31

FDA Drug

Yes

Countries

• United States
• Australia
• Canada

Study Locations