Panelists discuss how the updated NCCN Guidelines now recommend tazemetostat for the treatment of advanced epithelioid sarcoma, regardless of EZH2 mutation status, based on clinical trial data showing significant clinical benefits and improved progression-free survival in patients with both mutated and non-mutated disease.
Summary for Physicians:
NCCN Guidelines have recently been updated to recommend tazemetostat for the treatment of patients with epithelioid sarcoma, irrespective of EZH2 mutation status. Tazemetostat is an EZH2 inhibitor that works by targeting EZH2, an enzyme involved in the methylation of histone proteins, leading to the silencing of tumor suppressor genes. By inhibiting EZH2, tazemetostat helps to restore normal gene expression and suppresses tumor growth.
The guideline update is supported by data from clinical trials, specifically the EZH-202 study (NCT02601950), which demonstrated significant clinical benefit with tazemetostat in patients with advanced epithelioid sarcoma. Notably, the data showed durable responses and improved progression-free survival in patients with and without EZH2 mutations, supporting the inclusion of all patients, not just those with mutations, in treatment recommendations. This expansion is intended to provide broader access to this effective treatment for patients with this rare and aggressive sarcoma.
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