Nassar, Amin H.El-Am, EdwardDenu, RyanAlaiwi, Sarah AbouEl Zarif, TalalMacaron, WalidAbdel-Wahab, NohaDesai, AakashSmith, CalebParikh, KaushalAbbasi, MuhannadFarhat, Elias BouWilliams, James M.Collins, Jeremy D.Al-Hader, AhmadMcKay, Rana R.Malvar, CarmelSabra, MohamadZhong, CaiweiEl Alam, RaquelleChehab, OmarLima, JoaoPhan, MinhPria, Hanna Ferreira DallaTrevino, AlexandraNeilan, Tomas G.Kwan, Jennifer M.Ravi, VinodDeshpande, HariDemetri, GeorgeChoueiri, Toni K.Naqash, Abdul Rafeh2024-06-262024-06-262024-01-16Nassar AH, El-Am E, Denu R, et al. Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter Retrospective Study. JACC CardioOncol. 2024;6(1):71-79. Published 2024 Jan 16. doi:10.1016/j.jaccao.2023.11.007https://hdl.handle.net/1805/41903Background: Primary cardiac soft tissue sarcomas (CSTS) affect young adults, with dismal outcomes. Objectives: The aim of this study was to investigate the clinical outcomes of patients with CSTS receiving immune checkpoint inhibitors (ICIs). Methods: A retrospective, multi-institutional cohort study was conducted among patients with CSTS between 2015 and 2022. The patients were treated with ICI-based regimens. The Kaplan-Meier method was used to estimate overall survival (OS) and progression-free survival (PFS). Objective response rates were determined according to Response Evaluation Criteria in Solid Tumors version 1.1. Treatment-related adverse events were graded per the Common Terminology Criteria for Adverse Events version 5.0. Results: Among 24 patients with CSTS, 17 (70.8%) were White, and 13 (54.2%) were male. Eight patients (33.3%) had angiosarcoma. At the time of ICI treatment, 18 patients (75.0%) had metastatic CSTS, and 4 (16.7%) had locally advanced disease. ICIs were administered as the first-line therapy in 6 patients (25.0%) and as the second-line therapy or beyond in 18 patients (75.0%). For the 18 patients with available response data, objective response rate was 11.1% (n = 2 of 18). The median PFS and median OS in advanced and metastatic CSTS (n = 22) were 5.7 months (95% CI: 2.8-13.3 months) and 14.9 months (95% CI: 5.7-23.7 months), respectively. The median PFS and OS were significantly shorter in patients with cardiac angiosarcomas than in those with nonangiosarcoma CSTS: median PFS was 1.7 vs 11 months, respectively (P < 0.0001), and median OS was 3.0 vs 24.0 months, respectively (P = 0.008). Any grade treatment-related adverse events occurred exclusively in the 15 patients with nonangiosarcoma CSTS (n = 7 [46.7%]), of which 6 (40.0%) were grade ≥3. Conclusions: Although ICIs demonstrate modest activity in CSTS, durable benefit was observed in a subset of patients with nonangiosarcoma, albeit with higher toxicity.en-USAttribution-NonCommercial-NoDerivatives 4.0 InternationalCardiac sarcomasCardiac tumorsImmune checkpoint inhibitorsTreatment-related adverse eventsArticle