Malignant fibrous histiocytoma of the left ventricle of the heart

Clinical History

A patient was admitted to the hospital because he complained of having epigastric pain, dyspnea, fatigue, and loss of appetite for several weeks and dizziness once in a while. A chest X-ray, transthoracic echocardiography and helical CT examination were performed.

Imaging Findings

A 28-year-old male was admitted to the hospital because he complained of having epigastric pain, dyspnea, fatigue, and loss of appetite for several weeks and dizziness once in a while. His pulse rate was 95 per minute, and his blood pressure was 120/70 mmHg. On physical examination, a grade I/VI systolic murmur was heard at the lower left sternal border. His chest X-ray photographs revealed cardiomegaly and a prominent left atrial appendage. A transthoracic echocardiography examination was performed, and a multicystic mass was seen in his pericardium (Fig. 1). A contrast-enhanced helical CT examination (Fig. 2) was performed, and a mass adjacent to the great vessels and the left ventricle, invading the left atrium and the ventricle was seen. The mass was mostly cystic and began from the level of aortic arch and descended to the bottom of the left ventricle. It appeared that the mass was invading the cranial part of the left ventricular anterior and lateral wall, anterolateral part of the left atrial wall and the left atrial appendage, and was extending to the front of the main pulmonary artery outlet and the left ventricle, the main pulmonary artery, the root of the aorta and the right atrium crossing under the main pulmonary artery, and the space under the main carina and the left main bronchus. MRI was also performed. But it was found to be nondiagnostic because of motion artifacts. The patient was operated on. While the operation was being done, a fragile, easily bleeding and soft mass invading the main pulmonary artery, the outflow of the right ventricle and the wall of the left ventricle in the pericardium, was seen. The mass was 10x16 cm in diameter. There was no pleural invasion. It was not possible to excise the mass. A biopsy was taken for histological examination, and the operation was finalized without total resection. The reports confirm that the patient had a malignant fibrous histiocytoma (MFH), and he was made to undergo radiotherapy.

Discussion

An MFH refers to a soft tissue sarcoma and constitutes less than 3% of all primary intracardiac tumors. It is most frequently located intracardiacally in the left atrium as is classically seen in younger patients, predominantly women. Because of its localization in the heart, the impairment of the cardiac output leads to symptoms such as dyspnea, chest pain, and congestive heart failure. Cardiac MFH may be confused clinically and histologically with benign atrial myxoma, the most commonly found primary cardiac tumor. Because of its high aggressiveness, the average survival time is usually less than 12 months. Our patient had some cardiac symptoms and was young but was not a female. We expected the patient to survive only for 6–12 months because of invasiveness of the tumor and its inability to be resected. A clinical and radiological examination can help appropriately identify the nature of the tumor. Transesophageal echocardiography, helical computed tomography and magnetic resonance imaging are highly reliable techniques that help in detection of intracardiac tumors. But a definite diagnosis requires either immunohistochemical or ultrastructural confirmation. Radiologically, the tumor may appear as a nonhomogeneous, solid mass with necrotic and cystic parts. In our case, the tumor was found to be mostly cystic.

Differential Diagnosis List

Final Diagnosis

Cardiac malignant fibrous histiocytoma.

URL:	https://eurorad.org/case/3133
DOI:	10.1594/EURORAD/CASE.3133
ISSN:	1563-4086