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 AlamOmar 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

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)71-79
Number of pages9
JournalJACC: CardioOncology
Volume6
Issue number1
DOIs
StatePublished - Feb 2024

Keywords

  • cardiac sarcomas
  • cardiac tumors
  • immune checkpoint inhibitors
  • treatment-related adverse events

ASJC Scopus subject areas

  • Oncology
  • Cardiology and Cardiovascular Medicine

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