ICB was administered in 12 patients with SMARCA4/B1-altered tumors (1st line pembrolizumab in monotherapy or in combination in 83% for metastatic disease). Median PFS for all immunotherapy-treated patients was 360 days(95%CI 158-562)….In our cohort, patients with genetic alterations in SMARCA4/SMARCB1 genes presented high TMB and prolonged responses to immunotherapy.