Key result
Multimodal imaging resolves discrepant echocardiography and MRI findings to diagnose rare primary cardiac leiomyosarcoma.
Why the study?
Primary malignant cardiac tumors are extremely rare, and cardiac leiomyosarcoma rarely presents with functional severe mitral stenosis.
Case Report (n=1)
This case demonstrates the critical role of multimodal imaging, including the novel use of ultrasound contrast agents, in diagnosing rare primary cardiac leiomyosarcomas.
Multimodal imaging may resolve discrepancies in cardiac masses; extends sparse evidence on primary leiomyosarcoma presentations.
BACKGROUND: Primary malignant cardiac tumors are extremely rare (<0.03% prevalence). Cardiac leiomyosarcoma is especially uncommon and rarely presents with functional severe mitral stenosis. CASE SUMMARY: A 49-year-old man presented with worsening exertional chest pain and dyspnea. Imaging was discrepant between a malignant or benign mass. Magnetic resonance imaging (MRI) favored a benign mass, whereas echocardiography favored malignancy. Final pathology was consistent with high-grade left atrial leiomyosarcoma with myxoid features. DISCUSSION: Leiomyosarcomas are very rare primary malignancy cardiac tumors, and current literature consists mostly of case reports. Despite cardiac MRI being the gold standard for imaging cardiac masses, information gathered from echocardiography is crucial for both diagnosis and surgical planning. TAKE-HOME MESSAGES: General patterns of leiomyosarcoma include a large sessile left atrial mass, mixed hyperintense T1 and T2 signaling on MRI, and occurrence from the fourth to sixth decades of life. Careful consideration of all imaging findings is important, especially in instances where 1 modality suggests a benign mass and another suggests malignancy.
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Shakfeh et al. (2026) conducted a case report in Cardiac leiomyosarcoma (n=1). Multimodal imaging and surgical resection was evaluated. A rare primary cardiac leiomyosarcoma presenting as chest pain and severe mitral stenosis was diagnosed using a multimodal imaging approach, despite discrepant MRI and echocardiography findings.
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