Dr. Chi on Tazemetostat in Pediatric Patients 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 the EZH2 inhibitor tazemetostat in pediatric patients with INI1-negative tumors.

In a phase I study of pediatric patients with INI1-negative rhabdoid tumors, epithelioid sarcoma, chordoma, and synovial sarcoma tumors, tazemetostat showed promising antitumor activity. Chi reports that the drug was very well-tolerated, and the side effects seen were nausea, vomiting, and a small amount of bone marrow suppression. Tolerability is important for these children, as they have previously undergone intensive regimens of chemotherapy and radiation, Chi adds.

There was 1 case of a secondary malignancy. Chi says that she and her fellow investigators are currently evaluating this case. They are working on determining the relationship with the inhibitor and how it can be prevented in the future. Still, these findings have led to a phase II trial, where tazemetostat will be given at a recommended dose of 1200 mg/m² divided twice daily.