75 patients were evaluated with PFS/OS (months) results of 6.0/N.D. for the 600 mg/m2 group (n = 31), 7.2/22.5 for leiomyosarcoma (LMS) (n = 33), 5.1/17.4 for liposarcoma, and 2.8/15.0 for other (n = 36). Out of 75 patients, 8% (6/75) had CR (3 LMS, 1 synovial, and 2 angiosarcoma), 17% (13/75) had PR, and 43% (32/75) had SD, leading to an ORR of 25% (19/75) and a clinical benefit rate of 68% (51/75). There was a tendency for ASS1 negative tumors to benefit more than ASS1 positive tumors. Here, ASS1 is linked to leiomyosarcoma.