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PEPN2121: Tiragolumab and Atezolizumab for SMARCB1- or SMARCA4-deficient Tumors

About this study

This study will look at how well 2 medicines treat SMARCB1- and SMARCA4-deficient tumors. The cells in SMARCB1- and SMARCA4-deficient tumors lack SMARCB1 and SMARCA4 genes. These genes are present in certain types of cancers that are hard to treat.

The medicines in this study are tiragolumab and atezolizumab. They are a type of immunotherapy, which helps the body’s own immune system fight cancer. 

We will study how well these drugs work in children and young adults whose cancer has come back or did not respond to treatment.

There are 2 phases, or parts, to this clinical trial:

  • Phase 1 (or Part A) helps us decide the best dose of tiragolumab to use in children. Patients enrolled in phase 1 may be treated with tiragolumab along with atezolizumab at a later time. Once a safe dose is found in phase 1, these patients can enroll in phase 2 of the clinical trial.
  • Phase 2 (or Part B) is open to people 18 years and older. We will study how well tiragolumab and atezolizumab work in treating 6 kinds of cancer in young adults:
    • Renal medullary carcinoma
    • Malignant rhabdoid tumor
    • Atypical teratoid rhabdoid tumor
    • Poorly differentiated chordoma
    • Epithelioid sarcoma
    • Other SMARCB1- or SMARCA4-deficient tumors

Purpose of this clinical trial

  • Find the best dose of tiragolumab to use in children.
  • Determine how well tiragolumab and atezolizumab treat children, teens, and young adults with SMARCB1- or SMARCA4-deficient tumors that have come back or did not respond to therapy.

Eligibility overview

To take part in this study, you must:

  • Have a SMARCB1- or SMARCA4-deficient tumor that has come back or did not respond to therapy
  • Be 1–17 years old for phase 1
  • Be 18 years old or older for phase 2

The above information is intended to provide only a basic description about a research protocol that may be currently active at St. Jude. The details made available here may not be the most up-to-date information on protocols used by St. Jude. To receive full details about a protocol and its status and or use at St. Jude, a physician must contact St. Jude directly.


Full title:

PEPN2121: A Phase 1/2 Study of Tiragolumab and Atezolizumab in Patients with Relapsed or Refractory SMARCB1- or SMARCA4-deficient Tumors

Study goal:

Tiragolumab and atezolizumab will be used to treat pediatric and young adult patients with SMARCB1- and SMARCA4-deficient tumors.


Atypical teratoid/rhabdoid tumor, Chordoma, Epithelioid sarcoma, Kidney medullary carcinoma, Malignant solid neoplasm, Rhabdoid tumors


Phase 1: ages 1–17 years; Phase 2: age 18 and older

For physicians and researchers

Patients accepted to St. Jude must be referred by a physician or other qualified medical professional. Learn how St. Jude can partner with you to care for your patient.


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