Introduction: Primary cardiac myxofibrosarcoma (MFS) is a rare and aggressive malignancy, with limited treatment options and a high recurrence rate. Multimodal imaging is critical for diagnosis, and surgery remains the primary treatment, though recurrence is common. Case Presentation: We present the case of a 41-year-old male with history of intermittent chest pain and palpitations. Initial work-up revealed a left atrial mass, which after thorough evaluation with multimodal imaging and histopathological assessment, was diagnosed as a primary cardiac myxofibrosarcoma. Despite complete surgical resection and low-grade histology, recurrence ensued, prompting reintervention and adjuvant radiotherapy. Discussion: Cardiac MFS poses significant diagnostic challenges. Multimodal imaging and immunohistochemistry are key to diagnosis. While surgery is the mainstay of treatment, high recurrence rates necessitate consideration of adjuvant therapies and long-term monitoring. Conclusion: This case highlights the importance of differential diagnosis of intracardiac masses, as well as reaching a consensus regarding the approach for malignant cardiac tumors.
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