PMC10173306_01
Chest computed tomography demonstrated cardiac mass of Case1. (A) Chest x-ray and Chest CT images in axial (B) and coronal planes (C) showed remarkable extensive pulmonary lesions. (D) Contrast-enhanced CT showed a hypodense oval filling contrast defect (arrow) at the right atrium (RA). (E) 18F-fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) showed more uptake in the RA mass (arrow), lung, and pleura. (F) Echocardiography depicted an oval-shaped mass (22 x 41 mm) (arrow) in the RA at the level of the superior vena cava ostium.
fcvm-10-1103918-g001.jpg
PMC10173306_01_fcvm-10-1103918-g001.jpg
F1
C-reactive protein (CRP) (33.4 mg/L), leucocyte count (12.7 x 109/L), pro-Brain natriuretic peptide level 406 pg/ml, D-dimer of 9840 ug/L, and tumor marker CA125 (312.1 U/ml). Chest X-ray (Figure 1A) and computed tomography (Figures 1B,C) revealed striking extensive pulmonary lesions, a large soft tissue mass in the right atrium (RA), and pleural effusion. Contrast-enhanced CT showed a hypodense oval filling contrast defect at the right atrium (RA) (Figure 1D) 18F-fluorodeoxyglucose positron emission tomography/CT revealed focal uptake in the RA mass (with SUVmax of 5.8), and scattered uptake in lung and pleura metastatic (Figure 1E). Echocardiogram showed a large mass (22 x 41 mm) in the RA invading the superior vena cava (Figure 1F).
Histological endomyocardial biopsy specimen of the right atrium and lung rapid deterioration of Case1. (A) Catheter for intracardiac echocardiography (arrowhead) and cardiac bioptome (arrow). (B) The intracardial echocardiography demonstrates the mass of the right atrium and well-targeted biopsy catheter (arrow) on the mass. (C-F) The representative histologic aspect of the mass. Immunohistochemical staining for CD31(C), CD34 (D), and ERG (E) marker in cytoplasmic. (F) Cells displaying epithelioid morphology, large nuclei, and ample cytoplasm (hematoxylin and eosin). (Original magnification 40X). (G,H) Axial and coronal contrast-enhanced CT scans showed rapid progression of lung metastases, and (I) recurrent bloody pleural effusion one month after hospitalization. The arrow indicates progressive RA mass.
fcvm-10-1103918-g002.jpg
PMC10173306_01_fcvm-10-1103918-g002.jpg
F2
Histopathology results (Figures 2C-F) revealed a spindle cell tumor, staining with CD34, CD31, and ERG expression, confirming the diagnosis of cardiac epithelioid hemangioendothelioma (EHE). Unfortunately, the patient's condition deteriorated quickly due to aggressive pulmonary lesions (Figures 2G-I), and he had severe dyspnea.
PMC10173306_02
Comparison of lung metastases and cardiac mass before and after chemotherapy of Case2. (A,B) Frontal and lateral Chest x-ray demonstrated extensive pulmonary lesions. (C) Chest CT at coronal view and (D) axial view revealed severe lung metastases. (E) Contrast-enhanced Chest CT reveals a mass lesion (arrow) along the superior and posterior right atrial wall without enhancement. After 5 courses of chemotherapy, the number of lung metastases (F,G) was less than before (C,D), and the lateral wall of the RA (H) has no occupied (arrow) compared to earlier (E).
fcvm-10-1103918-g003.jpg
PMC10173306_02_fcvm-10-1103918-g003.jpg
F3
Her chest x-ray (Figures 3A,B) and chest CT (Figures 3C,D) revealed multiple lung lesions. The size is about 55mm x 28mm x 36 mm (Figure 3E). Anlotinib day1-14 for targeted therapy). After 5 courses of chemotherapy, the lateral wall of the right atrium has no occupied, and the number of lung metastases was less than before (Figures 3F-H).
Multimodality images and intracardiac echocardiography-guided biopsy of cardiac tumors of Case2. (A) Transthoracic echocardiography showed a huge mass lesion in the RA. (B) Cardiac enhanced-MRI revealed a large irregular mass (55.2 x 38.1 x 33.9 mm) with a rounded appearance in the RA and connected with the right atrium. (C) Catheter for intracardiac echocardiography (arrowhead) and cardiac bioptome(arrow). (D) The intracardial echocardiography demonstrates thickness mass of the right atrium and well-targeted biopsy catheter (arrow) on the mass. (E) Histopathological diagnosis revealed cardiac angiosarcoma, with spindle tumor cells.
fcvm-10-1103918-g004.jpg
PMC10173306_02_fcvm-10-1103918-g004.jpg
F4
Transthoracic echocardiography (Figure 4A) and contrast-enhanced Cardiac magnetic resonance (CMR) imaging (Figure 4B) confirmed a large irregular mass (55.2 x 38.1 x 33.9 mm) in the right atrium (RA) invading the superior vena cava. Histopathological results (Figure 4E) confirmed the diagnosis of cardiac angiosarcoma, with spindle tumor cells staining positively for CD34, CD31, Fli-1, and ERG, with ki-67 proliferation index reached 60%.
PMC10173306_03
Multiple investigations for diagnosis of Case3. (A) Transthoracic echocardiography showed cardiac mass lesion (arrow) in the RA. (B) Chest enhanced CT, and (C) Cardiac enhanced-MRI revealed a large mass (arrow) at the junction of the RA and RV. (D) Catheter for intracardiac echocardiography (arrowhead) and cardiac bioptome (arrow). (E) The intracardial echocardiography demonstrates well-targeted biopsy catheter (arrow) on the mass. (F) Histopathological imaging revealed small vascular lumen formed by single cell, and cardiac epithelioid hemangioendothelioma (EHE) was diagnosed.
fcvm-10-1103918-g005.jpg
PMC10173306_03_fcvm-10-1103918-g005.jpg
F5
Echocardiography showed a hyperechoic mass at the right ventricle lateral wall junction (Figure 5A). Enhanced-CMR and chest CT clearly confirmed that a mass (38mm x 23 mm) was located at the junction of the right atrium and ventricle and had a modest amount of pericardial effusion (Figures 5B,C). The biopsy technique was the same as above without complications (Figure 5D,E). This result revealed small vascular lumen formed by single cell (Figure 5F). and considered as an intermediate or low-grade malignant vascular tumor with positive CD34, CD31, Fli-1, and ERG expression, confirming the diagnosis of cardiac epithelioid hemangioendothelioma (EHE).