Dr. Chi on Response to Tazemetostat in Children With INI1-Negative Tumors

Susan N. Chi, MD, senior physician, director, Pediatric Brain Tumor Clinical Trials Program, Dana-Farber Cancer Institute, assistant professor of pediatrics, Harvard Medical School, discusses response to tazemetostat in children with INI1-negative tumors.

The EZH2 inhibitor tazemetostat showed promising antitumor activity in a phase I study of pediatric patients with INI1-negative rhabdoid tumors, epithelioid sarcoma, chordoma, and synovial sarcoma tumors. This phase I study showed several responses, which are not commonly seen this early, Chi says. These responses included complete responses (CRs). Three patients had a CR, while 1 had a partial response, and 5 had stable disease. These findings, Chi says, are encouraging moving into a phase II study.

Additionally, Chi notes that a company investing their time, energy, and money into a small pediatric study of a rare tumor type such as rhabdoid tumors or INI1-deficient tumors is encouraging. This small experimental study was completed with a relatively quick turnaround time, she adds.