Article

Characterization of Cardiac Sarcoma With 2-and 3-Dimensional Echocardiography, Myocardial Contrast Echocardiography and Cardiac Magnetic Resonance Imaging

Authors:
  • Barts Heart Centre, St Bartholomew's Hospital, London, UK
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Abstract

A 57-year–old woman had previously undergone a right hemicolectomy for Duke B adenocarcinoma of caecum. She presented with acute back pain. A magnetic resonance imaging scan of her thoracic spine showed destruction of a vertebra with a tumor deposit at T11 (Figure 1A). She underwent a course of radiotherapy (20 Gray in 5 fractions) to this area. Good symptomatic relief was obtained. Biopsy demonstrated a sarcomatoid tumor suggestive of leiomyosarcoma. Over the next 2 months she developed dyspnea on exertion, fatigue, and leg edema. Examination revealed a regular tachycardia, raised jugular venous pressure, and bilateral leg edema. On auscultation, an added early diastolic sound (consistent with tumor plop) was audible. A 3-dimensional echocardiogram identified a large mass protruding from the right atrium into the right ventricle (Figure 1B and online-only Data Supplement Movie I). The mass prolapsed through the tricuspid valve into the right ventricle during diastole, intermittently obstructing right ventricular inflow and almost completely occluding the tricuspid valve …

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... Differential diagnosis of a right atrial mass includes benign entities such as myxoma and thrombus versus malignant pathologies such as metastatic involvement of the heart, PCA, and primary cardiac lymphoma [2]. Cardiac tumor evaluation should combine imaging modalities that enable comprehensive evaluation of both anatomical as well as functional properties of the tumors [1][2][3]. ...
... PCA has a tendency to occur in the third to fifth decade and is more common in males. Although dyspnea is the most common symptom, symptoms vary according to site and size of tumor, myocardial involvement, and development of pericardial effusion [1][2][3]. The right atrium was reported to be the most affected site by PCA with invasion of the lateral (free) wall of the right atrium while the septum remains spared in most cases [1][2][3]. ...
... Although dyspnea is the most common symptom, symptoms vary according to site and size of tumor, myocardial involvement, and development of pericardial effusion [1][2][3]. The right atrium was reported to be the most affected site by PCA with invasion of the lateral (free) wall of the right atrium while the septum remains spared in most cases [1][2][3]. The differential diagnosis of a right atrial mass includes benign entities such as myxoma and thrombus versus malignant pathologies such as metastatic involvement of the heart, PCA, and primary cardiac lymphoma [2]. ...
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... Myocardial deformation echocardiography can be used to differentiate normally contracting myocardium from fibrotic tissue. The 3D echocardiography and transesophageal echocardiography can be used to better characterize the location and relationship of the tumor to other cardiac structures [6][7][8][9]. Transesophageal echocardiography is particularly useful for visualizing tumors located in the atria when the exact site of attachment is unclear from TTE and in assessing their relationship with the venae cavae [10]. Cardiac magnetic resonance (CMR) imaging may provide incremental information by not only assessing tumor anatomy but also tissue characterization of the mass. ...
... Echocardiography usually demonstrates an echogenic, nodular mass. As they are usually highly vascular, application of contrast agents may be used to demonstrate perfusion of the tumor with microbubbles (FIGURE 5) [6]. Pericardial involvement may occur by direct invasion or through formation of a pericardial effusion. ...
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Evaluation of cardiac tumors with cardiovascular imaging aims to establish aetiology, identify complications of tumor and help define management strategy. 2D echocardiography remains the primary diagnostic modality. Additional use of newer echocardiographic techniques such as 3D, strain and contrast echocardiography better characterise tumor morphology, tissue characteristics and vascularity respectively. Cardiac MRI and computed tomography provide complementary information and are able to identify extra-cardiac infiltration and also provide further tissue characterisation. This review explores the non-invasive diagnostic approach to evaluation of cardiac tumors.
... Use of TEE played a prominent role in establishing a PAS diagnosis in 2 other cases. 11,12 However, in these instances, TEE was used in the preoperative setting. Compared to other imaging modalities, TEE allows for the real-time characterization of the physiologic details of a lesion while simultaneously assessing ventricular and valvular function. ...
Article
Primary cardiac sarcomas are exceptionally rare. We present a 10-year, single-institution experience with 24 primary adult cardiac sarcomas. These cases were retrieved from the Department of Pathology data file of the Methodist Hospital at Houston, TX. Clinical presentation and pathologic features were analyzed. Histologic classification was followed according to the criteria set by the World Health Organization, and grading according to the system proposed by the Federation Nationale des Centres de Lutte Contrele Cancer. There were 14 men and 10 women (male/female, 1.4:1) with a mean age of 42.2 years (range 20-68 years). The tumors involved the right atrium in 14 cases, left atrium in 6 cases, right ventricle in 2 cases, and left ventricle in 2 cases. The tumor size ranged from 2.0 to 17.0 cm (mean 7.2 cm), and, histologically, there were 10 angiosarcomas, 9 unclassified sarcomas, 3 synovial sarcomas, and 2 leiomyosarcomas. All 10 angiosarcomas originated from the right atrium, whereas 5 of the unclassified sarcomas were from the left atrium. Although cases were limited, no predilection site was found for the other histologic types. All tumors were graded as 2 (5 cases) or 3 (19 cases) in differentiation. The prognosis was poor with a median survival time of 25 months after diagnosis. The grade was not statistically significant on survival (P = .14). In conclusion, angiosarcoma and unclassified sarcomas are the most common sarcomas of the heart accounting for 76%, but rare tumors such as synovial sarcoma and leiomyosarcoma may also occur in this organ. The survival of cardiac sarcomas is poor.