Pronged Survival after Surgical Resection of a Primary Mitral Valve Sarcoma

Objectives: Primary sarcoma of the mitral valve is rare. A complete surgical resection should be attempted even though the accessibility and location of the tumor may provide surgical challenges. The prognosis of patients with a primary cardiac sarcoma is very poor because of their resistance to therapy. This report describes a 48-year-old man who underwent mitral valve sarcoma resection and has remained tumor free for 7 years.

Methods and Results: In 2008 a 47-year-old Caucasian ship's captain was diagnosed by his family doctor with a cardiac murmur. Two years later, echocardiography (performed in the context of physical examination required by his employment) showed a mitral valve tumor (22 × 15 mm) with moderate mitral insufficiency assumed to be a myxoma. On January 2010 echocardiography and computed tomography revealed an increase in tumor size (32 × 18 mm) that obstructed mitral flow. During surgery on 1 March 2010 intraoperative histopathology revealed a cellular spindle proliferation confirming the diagnosis of sarcoma. The tumor, the mitral leaflets and a wide resection of the atrial wall was performed. The atrial reconstruction was then performed using an extracellular matrix-derived Biopatch and a biological prosthetic valve was inserted. Subsequent histology revealed a high-grade sarcoma without tumor free border. Following surgery the patient received a combination of radiotherapy (30 × 2 Gy from 20 April 2010 until 02 June 2010) and seven weekly cycles of chemotherapy with Adriblastin. Follow-up in March 2017 included blood work. Computed tomography of chest and abdomen revealed no evidence of metastasis.

Conclusion: Cardiac sarcomas have a dismal prognosis; overall median survival being a mere 6 months. Resection of malignant sarcoma has been considered palliative, not contributing to long-term survival. Our case shows that a complete surgical resection appears to be the only mode of therapy to prolong survival.

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