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Epithelioid sarcoma is a rare soft tissue sarcoma characterized by SMARCB1/INI1 deficiency. Much attention has been paid to the selective EZH2 inhibitor tazemetostat, where other systemic treatments are generally ignored. To explore alternative treatment options, we studied the effects of irinotecan-based chemotherapy in a series of epithelioid sarcoma patients.

Inclusion Criteria:

• (1) Grade 2 or 3 ES confirmed histologically using the American Joint Committee on Cancer (AJCC) system ;
• (2) patients presented with measurable lesions using the Response Evaluation Criteria In Solid Tumors (RECIST1.1) and were not amenable to surgical resection or radiotherapy;
• (3) primary or secondary metastatic disease;
• (4) received more than two courses of the VIA regimen;
• (5) no concurrent treatment was given while on the VIA regimen;
• (6) follow-up information and evaluation after chemotherapy were available.

Exclusion Criteria:

• less than 4 cycles of treatment;
• medical records were not complete.