Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter Retrospective Study

Amin H Nassar; Edward El-Am; Ryan Denu; Sarah Abou Alaiwi; Talal El Zarif; Walid Macaron; Noha Abdel-Wahab; Aakash Desai; Caleb Smith; Kaushal Parikh; Muhannad Abbasi; Elias Bou Farhat; James M Williams; Jeremy D Collins; Ahmad Al-Hader; Rana R McKay; Carmel Malvar; Mohamad Sabra; Caiwei Zhong; Raquelle El Alam; Omar Chehab; Joao Lima; Minh Phan; Hanna Ferreira Dalla Pria; Alexandra Trevino; Tomas G Neilan; Jennifer M Kwan; Vinod Ravi; Hari Deshpande; George Demetri; Toni K Choueiri; Abdul Rafeh Naqash

doi:10.1016/j.jaccao.2023.11.007

Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter Retrospective Study

JACC CardioOncol. 2024 Jan 16;6(1):71-79. doi: 10.1016/j.jaccao.2023.11.007. eCollection 2024 Feb.

Authors

Amin H Nassar¹, Edward El-Am², Ryan Denu³, Sarah Abou Alaiwi⁴, Talal El Zarif⁴, Walid Macaron³, Noha Abdel-Wahab³, Aakash Desai⁵, Caleb Smith⁵, Kaushal Parikh⁵, Muhannad Abbasi², Elias Bou Farhat⁴, James M Williams⁶, Jeremy D Collins⁶, Ahmad Al-Hader⁷, Rana R McKay⁸, Carmel Malvar⁸, Mohamad Sabra⁷, Caiwei Zhong⁴, Raquelle El Alam⁴, Omar Chehab⁹, Joao Lima⁹, Minh Phan¹⁰, Hanna Ferreira Dalla Pria¹¹, Alexandra Trevino¹², Tomas G Neilan¹³, Jennifer M Kwan¹, Vinod Ravi³, Hari Deshpande¹, George Demetri⁴, Toni K Choueiri⁴, Abdul Rafeh Naqash¹⁰

Affiliations

¹ Yale University School of Medicine, New Haven, Connecticut, USA.
² Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA.
³ University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
⁴ Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
⁵ Division of Medical Oncology, Mayo Clinic, Rochester, Minnesota, USA.
⁶ Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA.
⁷ Department of Internal Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.
⁸ Moores Cancer Center, University of California-San Diego, La Jolla, California, USA.
⁹ Division of Cardiology, Department of Medicine, Johns Hopkins University, Baltimore Maryland, USA.
¹⁰ Stephenson Cancer Center, University of Oklahoma, Oklahoma City, Oklahoma, USA.
¹¹ Division of Diagnostic Imaging, MD Anderson Cancer Center, Houston, Texas, USA.
¹² Department of Internal Medicine, Northwestern University, Chicago, Illinois, USA.
¹³ Cardio-Oncology Program, Division of Cardiology, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts, USA.

Abstract

Background: 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.

Keywords: cardiac sarcomas; cardiac tumors; immune checkpoint inhibitors; treatment-related adverse events.