Excerpt

A 45-year-old woman presented to another hospital with nausea, vomiting, poor appetite, and unintentional weight loss over several months. Computed tomography showed multiple uterine myomas, the largest of which was 17 × 12 mm, a left atrial mass of 3 × 3 cm, and an ileal mass. The patient underwent an ileal mass resection owing to signs of mechanical obstruction, and pathology was consistent with leiomyosarcoma. Her follow-up was complicated by progressive dyspnea. She was transferred to our institution on the 18th postoperative day because echocardiography revealed an enlarged left atrial mass. On admission, the patient was alert and dyspneic. A physical examination showed that the patient was afebrile, had a blood pressure of 100/60 mmHg, pulse of 95 bpm, and oxygen saturation of 90 % on room air. Cardiac auscultation revealed tachycardia and grade 3/6 systolic and diastolic murmurs best heard at the cardiac apex. Fine crackles were heard at the lung bases. The patient’s electrocardiogram was unremarkable. Subsequent transthoracic and transesophageal echocardiography revealed a left atrial mass, which occupied most of the left atrium (Fig. 1). The mass was mobile, attached to the mitral valve, and protruded into the left ventricular cavity during diastole. The mass interfered with valve function, causing mitral stenosis (peak gradient: 36; mean gradient: 26 mmHg) and severe mitral regurgitation. A preoperative angiogram was normal with the exception of neovascularization of the tumor (Fig. 2). At this stage, no further diagnostic work-up was performed because of the urgent need for surgery. During surgery, an 11.5 × 5-cm mass was found to invade the anterior mitral leaflet, posterior wall of the left atrium, and left auricula (Fig. 3). The mass was completely excised along with the posterior wall of the atrium, the mitral valve was replaced with a prosthetic valve, and the left atrium and interatrial septum were reconstructed using a cardiac patch. Macroscopically, the tumor was a bulky, whitish-grayish mass that contained diverse necrotic and hemorrhagic areas and invaded the left atrial auricula. The surgical margin results were negative. The mass showed evidence of necrosis and had a mitotic index of 8/10 high-power fields. The tumor cells were immunohistochemically positive for muscle-specific actin and vimentin, with focal desmin positivity, were negative for panCK, CD34, and c-kit consistent with leiomyosarcoma (Fig. 4). The patient’s early postoperative follow-up was uneventful, and she was successfully extubated on day 2; however, she collapsed suddenly on day 5 because of a malignant ventricular arrhythmia and died despite all attempts to resuscitate her. …