Early clinical and metabolic response to tazemetostat in advanced relapsed INI1 negative epithelioid sarcoma.

Epithelioid sarcoma (ES) is a rare soft tissue sarcoma with an incidence of 0.05 per 100,000 population in the USA. It is characterized by multiple local recurrences and regional lymph nodes form the commonest site of metastases. The function of Integrase Inhibitor 1 (INI1) protein is lost in more than 90% of cases, which was the basis for the introduction of tazemetostat into the therapeutic armamentarium for management of advanced ES. The efficacy and manageable toxicity profile of tazemetostat have been demonstrated recently, leading to its accelerated approval for treatment of advanced ES. We report one of the first real-world cases of relapsed, metastatic ES treated with tazemetostat. The patient attained partial response with the therapy and is tolerating the drug well without serious toxicities.