Surgical resection of two independent primary intimal sarcomas in the left atrium

Primary intimal sarcoma of the heart is an extremely rare tumor that is known to have a very poor prognosis. We present a case of a 65-year-old man who suffered from deteriorating congestive heart failure due to a severe mitral stenosis caused by a large mobile left atrial tumor. The patient underwent an emergency operation of the tumor in the left atrium. The tumor was attached to the inferior wall of the left atrium. After the resection of the tumor, a second tumor on the interatrial septum, which had not been detected in the preoperative investigation, was discovered and resected. The patient developed acute respiratory failure soon after the operation and succumbed to his illness. The appearance of the main tumor was cauliflower-like, which strongly suggested the possibility of malignancy. Immunohistochemistry revealed that the neoplastic cells were positive for vimentin, desmin, p16, and especially murine double minute 2 (MDM-2). The first tumor was CD34 positive and cdk4 negative, but the second tumor was more anaplastic and CD34 negative and cdk4 positive, which suggests a different origin of the two tumors. The two tumors were diagnosed as intimal sarcomas by MDM-2, which is currently considered a conclusive marker. This is an exceptionally rare case of two simultaneous and possibly independent primary intimal sarcomas in the left atrium.