Background: ChRCC constitutes 5-10% of all RCC cases, and is generally associated with better prognosis including benefit from approved targeted agents (Armstrong, Lancet Onc, 2016). Presence of sarcomatoid features (SF) have previously been proposed as an adverse prognostic marker, but data for systemic therapy is limited. We assessed therapeutic outcomes for pts with metastatic ChRCC with and without SF. Methods: Retrospective chart reviews of pts with newly diagnosed metastatic ChRCC evaluated at Memorial Sloan Kettering Cancer Center (MSKCC) between 2002-17. MSKCC pathology reports determined presence/absence of SF to categorize pts. Evaluated endpoints included overall survival, time to treatment (TTF) for pts who initiated first line therapy at MSKCC and time to recurrence (TTR) post nephrectomy with metastatic disease. OS between the 2 groups was compared using log-rank analysis. A subset of pts had next generation sequencing (NGS) with MSK-IMPACT. Results: 109 pts with newly diagnosed metastatic ChRCC were identified (80 without SF, 29 with SF). Pts with SF were more likely to present with IMDC poor risk status (31% vs 10%) and de novo metastatic disease (48% vs 19%). 52 pts initiated first line therapy at MSKCC (35 without SF, 17 with SF). First line agents included VEGF tyrosine kinase inhibitors (60%), mTOR inhibitors (13%), cytokines (6%) and other investigational agents (21%). Outcomes are summarized in table below. NGS was performed in 22 pts, of which 64% and 45% harbored TP53 and PTEN alterations. Conclusions: Pts with metastatic ChRCC with SF had significantly worse outcomes compared to pts without SF. Median TTR < 3 months for this subgroup supports close surveillance following nephrectomy for localized tumors. The lack of benefit observed across various classes of systemic agents warrants study of underlying biology and investigating novel agents.