Abstract 95P
Background
Primary cardiac sarcomas are exceptionally rare neoplasms. Their nonspecific symptoms often lead to diagnosis at advanced stages. Complete surgical resection (R0) is the primary treatment goal; however, it is rarely achievable, resulting in high recurrence rates and poor survival outcomes.
Methods
We conducted a retrospective, observational, single-center study from February 2017 to December 2024 at a national referral center in Spain. Clinical, pathological, and therapeutic characteristics, as well as patient outcomes, were analyzed using data from medical charts. Descriptive and survival analyses were performed.
Results
We identified 12 cases of primary cardiac sarcoma. Median age was 43.4 years (range 13–76), and 58.3% were female. The most common symptom at diagnosis was dyspnea (n=8, 66.7%), and the right atrium was the most frequent tumor location (n=6, 50%). Angiosarcoma was the predominant histologic subtype (n=6, 50%). Localized disease was observed in 58.3% (n=7) of patients at diagnosis. Surgery was performed in 85.7% (n=6) of cases, but R0 resection was achieved in only 33.3% (n=2). 2 patients received adjuvant chemotherapy, and one underwent cardiac transplant. All patients experienced relapse, with a median recurrence-free survival of 5 months (CI 95% 1.5–8.6). Median overall survival (OS) for localized disease was 22.1 months (CI 95% 16.0–28.1). Metastatic disease was present in 41.7% (n=5) of patients at diagnosis, and 91.5% (11/12) developed metastases during their disease course. All metastatic patients received systemic treatment, with a median of 2 treatment lines. Median progression-free survival for first-line treatment (n=12) was 5.9 months (CI 95% 1.8–9.9), and OS for advanced disease was 12 months (CI 95% 10.3–13.6).
Conclusions
Despite most patients presenting with localized disease, complete surgical resection was rarely achieved, and recurrence was universal. Outcomes remain poor even for earlier stages. Our results highlight the limited efficacy of current therapies and the urgent need for improved multimodal approaches.
Clinical trial identification
Editorial acknowledgement
Legal entity responsible for the study
The authors.
Funding
Has not received any funding.
Disclosure
All authors have declared no conflicts of interest.