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Lead Investigator Discusses Tazemetostat Data in INI1-Negative Pediatric Tumors

Angelica Welch
Published: Wednesday, May 30, 2018

Susan N. Chi, MD

Susan N. Chi, MD
Tazemetostat showed promising antitumor activity in pediatric patients with INI1-negative rhabdoid tumors, epithelioid sarcoma, chordoma, and synovial sarcoma tumors, according to findings presented at the 2018 ASPHO Conference.

, Chi, director, Pediatric Neuro-Oncology and Clinical Trials, Dana-Farber Cancer Institute, discussed the rationale for using tazemetostat in this pediatric population, as well as the future steps being taken with this treatment.

OncLive: Can you provide an overview of the study?

Chi: I have a particular interest in patients with atypical teratoid rhabdoid tumors (ATRTs), which are very rare central nervous system tumors. This is a tumor that is distinct, in that it only has one specific mutation in its DNA in this gene called INI1 or SMARCB1. There are other tumors that have a very similar type of mutation, and those are rhabdoid tumors of the kidney and rhabdoid tumors of the soft tissues. Other tumors types include epithelioid sarcoma, renal cell medullary carcinoma, and chordomas. 
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