TriangleHEALTH PROFESSIONAL · SOURCE READING
Targeted therapy
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
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Evidence (tazemetostat):
In a phase II trial of 62 adult patients with epithelioid sarcoma and documented loss of INI1 by immunohistochemistry or biallelic SMARCB1 (the gene that encodes INI1) alterations, tazemetostat showed clinical activity.[62]
Source links and citations
In a phase II trial of 62 adult patients with epithelioid sarcoma and documented loss of INI1 by immunohistochemistry or biallelic SMARCB1 (the gene that encodes INI1) alterations, tazemetostat showed clinical activity.[62]
There were 9 of 62 confirmed partial responses, with an objective response rate of 15% and a disease control rate of 26%.
In January 2020, the U.S. Food and Drug Administration (FDA) granted accelerated approval to tazemetostat for adult and pediatric patients aged 16 years and older with metastatic or locally advanced epithelioid sarcoma who were not eligible for complete resection.
Publication references
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Preserved source evidence · Not medical advice