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.
