Primary cardiac myxofibrosarcoma: a rare case report

Introduction: Myxofibrosarcoma, historically considered as a subtype of MFH, is now recognized as a distinct entity according to WHO classification. It is a rare and aggressive soft tissue sarcoma of mesenchymal origin. Its occurrence as a primary cardiac tumor is exceptionally uncommon. Malignant tumors account for approximately 25% of all primary cardiac masses, with myxofibrosarcoma being particularly rare and posing diagnostic challenges due to nonspecific symptoms. Case presentation: We report a 42-year-old male presenting with progressive dyspnea and recurrent syncope. Echocardiography revealed a large left atrial mass partially obstructing the mitral valve. Surgical excision was performed, including removal of the posterior mitral leaflet and valve replacement with a 29-mm CARBOMEDICS mechanical prosthesis. Histopathology confirmed high-grade myxofibrosarcoma (Grade II), with immunohistochemistry positive for vimentin and negative for CD34, calretinin, desmin, and actin. The patient received adjuvant chemotherapy (ifosfamide and epirubicin). Despite treatment, he passed away 6 months postoperatively. Discussion: Primary cardiac myxofibrosarcoma is exceedingly rare and often presents with nonspecific symptoms such as dyspnea and syncope. Complete surgical excision remains the mainstay of treatment, although prognosis is generally poor. A literature review indicates very few reported cases, highlighting the rarity of this malignancy. Early recognition and intervention are essential to optimize patient outcomes. Conclusion: This case emphasizes the importance of timely diagnosis and management of primary cardiac myxofibrosarcoma. Early surgical intervention combined with adjuvant therapy may improve short-term outcomes. Further multicenter studies are needed to establish standardized treatment strategies for this aggressive tumor.