Progress in the management of primary and metastatic cardiac sarcoma: Advancing multidisciplinary care in an academic sarcoma center.

Abstract

e23568 Background: Cardiac sarcomas are rare tumors that are usually aggressive and associated with poor prognosis. While the optimal treatment strategy for cardiac sarcomas has yet to be established, a multidisciplinary approach including surgery, radiation and systemic therapies are utilized. We aimed to investigate the clinical characteristics, prognosis of cardiac sarcomas and the evolving treatment paradigms in this cohort. Methods: Pts diagnosed with primary and metastatic cardiac sarcomas were identified between January 2016 and 2025 at the Indiana University Cancer Center. Data was extracted from electronic medical records, including demographics, pathology, molecular testing, treatment and outcomes. Results: Eleven pts (5 female) were identified, with a median age at diagnosis of 38 years (range: 27-74). Dyspnea and chest pain were the most common presenting symptoms. The predominant histological subtypes were angiosarcomas of the right atrium and intimal sarcomas located in the left atrium or the left pulmonary artery. Other histopathologies included rhabdomyosarcoma, undifferentiated pleomorphic sarcoma, myxoid liposarcoma and chordoma. Six pts underwent upfront surgical resection of cardiac sarcomas: 4 with local disease underwent attempted curative surgery, but all had recurrence at a median time of 23.5 months (range: 5-80 months). Two pts with metastatic disease underwent definitive resection of both the cardiac tumor and all macroscopic disease, with 1 patient recurring at 52 months and the second remaining with no evidence of disease. Most pts (n = 5) required subsequent resection of primary cardiac recurrence and/or macroscopic disease, with 1 patient undergoing three sternotomies. Of the cohort, 10 pts received systemic therapy. Six pts received historical standard adjuvant doxorubicin based-chemotherapy, of which 5 subsequently received additional systemic treatment with an average of 3 lines of therapy (range: 1-7), including novel combinations of immunotherapy (n = 5) and targeted therapy (n = 4). Two pts achieved a complete response with surgery and adjuvant chemotherapy. One patient with metastatic primary cardiac intimal sarcoma had a complete response to immunotherapy. The median follow-up was 24 months (range: 3-120). Three pts died due to disease progression while four pts are alive with stable disease, three pts are alive with no evidence of disease, and one patient is alive with disease progression. Conclusions: Better outcomes were observed in those who underwent surgery and systemic therapy, findings that are consistent with previous studies. These findings underscore the complexity of managing cardiac sarcomas and the critical role of multidisciplinary care. Multi-institutional studies are needed to further elucidate comprehensive treatment strategies and biology-driven therapeutic regimens for these rare tumors.