Texas Children's Cancer and Hematology Center Tazemetostat + Nivolumab/Ipilimumab for SMARCB1-Negative or SMARCA4-Deficient Tumors (TAZNI)

A Phase I/II study testing a new drug combination for children and young adults with rare, aggressive tumors lacking SMARCB1 (INI1) or SMARCA4 proteins.

Description

SMARCB1 and SMARCA4 are proteins that help control how cells grow. Some rare and aggressive tumors—like malignant rhabdoid tumors, atypical teratoid rhabdoid tumors, epithelioid sarcoma, and chordoma—lack these proteins, which can lead to uncontrolled tumor growth.

This study is testing a combination of three drugs:

• Tazemetostat: a targeted therapy that blocks EZH2, a protein that helps cancer cells grow.
• Nivolumab and Ipilimumab: immunotherapy drugs that help activate the immune system to recognize and attack cancer cells.

The goal is to find the safest dose and see if this combination can effectively treat these tumors. The study includes blood and tissue sample collection for research, including biomarker analysis.

This trial is important because it offers a new approach for patients with these aggressive cancers, who have completed initial treatment and may not have other options. While these drugs have been used separately in children and adults, this combination has not yet been approved for these specific cancers.

Selected Eligibility

• Ages 6 months to 21 years
• Diagnosed with one of the following:
  • Atypical Teratoid Rhabdoid Tumor (ATRT)
  • Malignant Rhabdoid Tumor (MRT)
  • Rhabdoid Tumor of the Kidney (RTK)
  • Epithelioid Sarcoma
  • Chordoma (poorly differentiated or de-differentiated)
  • Other INI1- or SMARCA4-deficient tumors (with approval)
• Must have completed initial treatment
• Must meet performance and organ function criteria

Detailed inclusion and exclusion criteria are listed on clinicaltrials.gov