Abstract
We present the case of a 53 years-old woman presenting with congestive heart failure and pleural and pericardial effusions, in whom transthoracic and transesophageal echocardiography revealed multilocular cardiac tumor involving the left atrium wall, extending into the pericardium. Tumor was excised surgically and proved to be a malignant fibrous histiocytoma, primarily confined to the heart. Despite surgery followed by chemotherapy, the patient died a few months later. This is the 47th case of primary cardiac fibrous malignant histiocytoma reported in the literature. The pericardial involvement has been only rarely reported. Echocardiography was the method of diagnosis before rapid referral to surgery.
A 53-year-old woman was admitted to the hospital in October 2002 with severe dyspnea, chest pain and cyanosis. Symptoms of fatigue and dyspnea started 1 month before admission, with dyspnea at rest in the last 2 weeks. Clinical examination revealed severe congestive heart failure with bilateral hydrothorax, elevated jugular venous pressure, liver enlargement and peripheral edema. Blood pressure was 100/60mmHg, heart rate 110/min, and respiratory rate 24/min. On cardiac auscultation, an intermittent mitral diastolic rumble and a pericardial friction rub were heard. The ECG showed sinus tachycardia 110/min and low QRS voltages. X-ray showed an enlarged heart and bilateral pleural effusion.
Transthoracic echocardiography (TTE) on admission showed two echogenic masses – a 26/21mm mass attached to the posterolateral left atrial (LA) wall and a 24/30mm mass attached to the posterior left atrial wall. This mass was protruding into the left ventricle (LV) causing a transient obstruction between the LA and the LV. Massive pericardial effusion was present without signs of pericardial tamponade. A 17/38mm hyperechogenic mass was also identified in the pericardium near the lateral wall of LA and LV ( Fig. 1 A). The differential diagnosis was an atrial myxoma with multiple localizations or a malignant tumor which was more probable because of the pericardial effusion and the pericardial mass.
(A) Transthoracic echocardiography: the apical four-chamber view shows two echogenic masses (arrows), one fixed to the posterolateral LA wall and the second one protruded into the LV, with extension into the pericardium and pericardial effusion; (B) Multiplane transesophageal echocardiography: apical four-chamber view shows a multi-lobular atrial tumor with multiple sites of implantation that protrudes into the pulmonary veins and into the left ventricle.
(A) Transthoracic echocardiography: the apical four-chamber view shows two echogenic masses (arrows), one fixed to the posterolateral LA wall and the second one protruded into the LV, with extension into the pericardium and pericardial effusion; (B) Multiplane transesophageal echocardiography: apical four-chamber view shows a multi-lobular atrial tumor with multiple sites of implantation that protrudes into the pulmonary veins and into the left ventricle.
Transesophageal echocardiography (TEE) confirmed the presence of a multi-lobular atrial mass with multiple sites of implantation protruding into the pulmonary veins and into the mitral valve ( Fig. 1 B).
Spiral computer-tomography examination confirmed the echocardiographical findings. No other tumor was found in the chest or abdomen.
The patient was transferred to the cardiovascular surgery department and operated the next day. The atrial tumor and the pericardial mass that infiltrated the LA wall were excised and the pericardium was removed. Echocardiography after surgery was normal.
The histopathology performed during the operation diagnosed a fibro sarcoma but subsequently the imunohistochemistry examination proved the diagnosis of a malignant fibrous histiocytoma (MFH) ( Fig. 2 A and B). Chemotherapy was immediately initiated but a TTE performed 1 month later showed recurrence of the tumor both in the LA and the pericardium and pericardial effusion. The patient died in April 2003.
Immunohistochemical examination (tristadial ABC method). (A) Malignant fibrous histiocytoma (MFH), storiform–pleomorphic subtype. Fibrinous inflammation of the suprajacent endocardium. HE staining; ob. 5×. (B) MFH – numerous positive tumoral cells for F XIIIa in the storiform zone. Immunostaining for F XIIIa; ob. 10×.
Immunohistochemical examination (tristadial ABC method). (A) Malignant fibrous histiocytoma (MFH), storiform–pleomorphic subtype. Fibrinous inflammation of the suprajacent endocardium. HE staining; ob. 5×. (B) MFH – numerous positive tumoral cells for F XIIIa in the storiform zone. Immunostaining for F XIIIa; ob. 10×.
Discussion
To date, only 46 cases of primary cardiac MFH have been reported. 1 The frequency of MFH among primary cardiac sarcomas is 11.7%, making it the second most common primary cardiac sarcoma. MFH is a malignant tumor of fibroblasts and pleomorphic histiocytoid cells with large numbers of bizarre, multi-nucleated giant cells. 2
MFH most frequently originates in the LA, cardiac valvular involvement being found in as many as 50% of lesions. Because of this location, the symptoms of cardiac MFH are related to pulmonary congestion due to pulmonary vein obstruction, mitral stenosis, mitral regurgitation and right ventricular failure. The prognosis of intracardiac MFH is poor. Pericardial invasion rarely occurs.
Echocardiography is the primary tool for the diagnosis of intracardiac mass lesions, 3,4 but the histological study of the operative specimen is necessary for the histology type of the tumor. Improvement in cardiac imaging techniques has considerably increased the number of patients with a primary cardiac tumor referred for surgical excision. 5 In our case, CT did not reveal additional information regarding the characteristics of the intracardiac tumor.
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