A Phase 1-2 Study of Tazemetostat, Nivolumab, and Ipilimumab Immunotherapy in Young People With Tumors Missing the SMARCB1 or SMARCA4 Protein

Full Title

TAZNI: A Phase I/II Combination Trial of Tazemetostat with Nivolumab and Ipilimumab for Children with SMARCB1- or SMARCA4-Deficient Tumors

Purpose

Researchers are assessing different doses of tazemetostat given with nivolumab and ipilimumab in children, adolescents, and young adults with cancer. The patients in this study have a tumor that is missing the SMARCB1 or SMARCA4 protein. They have previously received treatment for the tumor.

The SMARCB1 and SMARCA4 proteins help prevent the uncontrolled growth of cells that can lead to cancer. SMARCB1- and SMARCA4-deficient tumors are aggressive and rare. They can arise in the kidney, soft tissues, central nervous system, and nasal cavity and sinuses. The people in this study have SMARCB1- or SMARCA4-deficient tumors such as:

  • Atypical teratoid rhabdoid tumor
  • Malignant rhabdoid tumor
  • Rhabdoid tumor of the kidney
  • Epithelioid sarcoma
  • Chordoma

Tazemetostat targets and kills some kinds of cancer cells by blocking a protein called EZH2. This protein controls which growth signals to cells are turned on and which are turned off. Tazemetostat is taken orally (by mouth).

Nivolumab and ipilimumab boost the power of the immune system to find and kill cancer cells. They are given intravenously (by vein).

Who Can Join

To join this study, there are a few conditions. You or your child must:

  • Have one of the tumors listed above or another SMARCB1- or SMARCA4-deficient tumor.
  • Have completed initial cancer treatment.
  • Have recovered from the serious side effects of prior therapies before getting the study treatment.
  • Be age 6 months to 21 years.

Contact

For more information or to see if you or your child can join this study, please call 833-MSK-KIDS.

Protocol

25-287

Phase

Phase I/II (phases 1 and 2 combined)

Investigator

Co-Investigators

ClinicalTrials.gov ID

NCT05407441