INTRODUCTION:The heart is commonly involved in various diseases with the exception of malignancies, especially primary cardiac tumors. We report a 22 year old man with a high grade, poorly differentiated sarcoma arising from and occluding the cavity of the left atrium.
CASE PRESENTATION:A 22 year old college student, with a past medical history of patent foramen ovale, was admitted to the ICU for complains of malaise, fatigue and shortness of breath, progressively getting worse since 4 days. He had also developed a cough, productive of orange, frothy sputum. His condition deteriorated to the point where he required intubation for respiratory fatigue and desaturation. CT scan of the thorax showed bilateral pulmonary infiltrates, consistent with edema, and moderate bilateral pleural effusions. He was initially started on broad spectrum antibiotics, including fungal coverage. An echocardiogram was performed which showed a mass in the left atrium. Transesophageal echocardiogram followed, showing a 4.7 cm × 4 cm mass in the left atrium attached to the posterosuperior wall. Cardiothoracic surgery was consulted and the mass removed. It weighed 36.5 grams and the actual size was 7cm × 5.2cm × 4.1cm. Grossly, it appeared tan white, and firm with vaguely nodular and focal gelatinous areas. Microscopic sections were consistent with a poorly differentiated, high grade sarcoma, with pleomorphic epitheloid cells, and few scattered multinucleated giant cell forms within a loose stroma. There were prominent areas of vascular invasion and necrosis. Immunohistochemical stains were negative and therefore comments on further classification were not possible. Florescence in situ hybridization was negative for SYT gene break-apart rearrangement, ruling out the synovial variety.Following surgery the patient was extubated within 24 hours. Repeat CT scan showed no edema with decreasing effusions. The patient was discharged 3 days later.
DISCUSSIONS:Cardiac tumors are mostly benign. Only 25% are malignant, most common of which are cardiac sarcomas. They are of several different histological types; the most common of which is the angiosarcoma. It also has the worst prognosis. There is no defined age for cardiac sarcomas, and the range is anywhere between 1 and 75 years, depending on the histology. They manifest by one of the following mechanisms:1. Obstruction to blood flow and interference with valve function2. Local invasion causing arrhythmias or pericardial effusion with or without tamponade3. Embolic phenomenonSymptoms include dyspnea, syncope, chest pain, fever, malaise or weight loss. Even if all symptoms are present a high index of suspicion is required to perform the right diagnostic tests. Initial diagnostic study of choice is a transthoracic or a transesophageal echocardiogram. Once the mass is seen, CT scan and MRI help to distinguish between tumors and thrombus. They visualize the extent of local invasion, compression of cardiac chambers, and involvement of the great vessels and the pericardium. They also give clues, which help to distinguish between benign and malignant lesions. MRI can further assess the tumor burden, volume and extent of mediastinal invasion. Definitive diagnosis can only be obtained from biopsy specimens. The information obtained from the imaging studies is prudent before surgery to perform an adequate resection, which is the only hope for long term survival. Chemotherapy and radiation have not proven beneficial so far.
CONCLUSION:Although this patient presented with classic symptoms of a cardiac tumor, the rarity of this condition precluded its presence in the initial diagnostic work up. The importance of cardiac imaging should not be undermined, as complete surgical resection remains the only hope for long term survival in these patients.
DISCLOSURE:Ali Kanchwala, None.