Background: Primary cardiac sarcomas are exceedingly rare tumours associated with a poor prognosis due to delayed diagnosis, advanced presentation and limited known chemotherapy efficacy. While surgical excision is the preferred treatment, it is often not feasible. The role of systemic therapy remains uncertain.
Methods: We analysed the medical records of patients diagnosed with primary cardiac sarcoma registered at a sarcoma clinic between January 2016 and July 2023. Clinicopathological and imaging data and treatment information were collected. Descriptive statistics were employed for clinicopathological characteristics, and Kaplan–Meier analysis was used for survival assessment.
Results: A total of 12 patients were identified with primary cardiac sarcoma, with a median age at diagnosis of 33 years (IQR 20.5; range: 17–53). At presentation, 66.7% had pericardial effusion requiring pericardiocentesis with or without pleuropericardial window. Half (6 of 12 patients) were misdiagnosed initially as either tubercular pericardial effusion or cardiac hydatid cyst. Upfront resection was done for 4 patients (33.3%), while 4 (33.3%) had locally advanced/unresectable disease and the remaining 4 (33.3%) presented with de-novo metastatic disease. Angiosarcoma constituted 50% (6 out of 12), all arising from the right atrium. Of the 12 patients included, 6 received a median two lines of therapy. Of the total 9 response assessments (both as first line and subsequent lines), 55.5% had an objective response rate with an 88.8% clinical benefit rate. The median progression-free survival for first-line systemic therapy was 5.4 months. The median overall survival for patients who received systemic therapy and the entire cohort were 19.2 and 5.1 months, respectively.
Conclusion: This study highlights the advanced presentation and poor outcomes in patients with cardiac sarcoma. Due to the rarity of the tumour, it is often misdiagnosed. Systemic chemotherapy could alleviate symptoms and prolong survival. However, sarcoma pathology is heterogenous and cannot be generalised.
Trial registration: As this is a retrospective observational study, no trial registration has been done.
