Stacchiotti S, et al. - Researchers tested pazopanib (an antiangiogenic drug) with respect to its activity in patients with advanced extraskeletal myxoid chondrosarcoma enrolled in this open-label phase 2 trial. They administered oral pazopanib (800 mg/day) continuously to participants, until disease progression, unacceptable toxicity, death, non-compliance, patient refusal, or investigator's decision. Patients who gave consent and had a central molecularly confirmed diagnosis of extraskeletal myxoid chondrosarcoma were included in the modified intention-to-treat population. Evaluation of the proportion of patients reaching an objective response according to Response Evaluation Criteria in Solid Tumors (RECIST) 1.1 in the modified intention-to-treat population was done (primary endpoint). There were 22 patients evaluable for the primary endpoint, and of these, 4 (18% [95% CI 1–36]) achieved a RECIST objective response during a median follow-up of 27 months (IQR 18–30). Overall, findings revealed the clinically meaningful antitumor activity of pazopanib in patients with progressive and advanced extraskeletal myxoid chondrosarcoma. In these patients, pazopanib could be thought of as a suitable choice following failure to respond to first-line anthracycline-based chemotherapy.