Epithelioid sarcoma is a rare type of cancer that starts as a growth of cells the soft tissue. It is more common in younger males.

Epithelioid sarcoma presents as a firm, painless, slow growing mass in the deep soft tissue or skin of the arms, hands, or fingers. It may also occur in the legs, trunk, or head and neck.

Epithelioid sarcoma involves the loss of Integrase Inhibitor 1 (INI1) protein expression. INI1 is a subunit of the SWI/SNF chromatin remodeling complex that opposes the enzymatic function of EZH2. When INI1 loses its regulatory function, EZH2 activity is de-regulated, allowing EZH2 to play a role cancer development.

Tazverik (tazemetostat) is FDA-approved for the treatment of adults and pediatric patients aged 16 years and older with metastatic or locally advanced epithelioid sarcoma not eligible for complete resection. It works as an EZH2 inhibitor.

Drugs used to treat Epithelioid Sarcoma