case_images: PMC11022687_01
case_images: Imaging findings. Plain radiography (A) showing the enlargement of left second and third arc protrusion and echocardiography (B) showed a mass on the lateral side of the left ventricle. Computed Tomography (CT) revealed cardiac enlargement, pericardial effusion, and bilateral pleural effusion (C). The enhanced CT showed tumor with faint enhancement and no calcification (D).
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case_images: Electrocardiography revealed normal sinus rhythm, and plain chest radiography revealed cardiomegaly in the left second and third arc protrusion ( Figure 1A ). Echocardiography revealed a mass on the lateral side of the left ventricle (LV) ( Figure 1B ). CT revealed cardiac enlargement, a large pericardial effusion, and bilateral pleural effusion ( Figure 1C ). No calcification, pericardial or thoracic dissemination or metastasis was found ( Figure 1D ). The results of immunohistochemistry (IHC) were as follows; SS18-SSX (-) ( Figure 4D ), SSX C-terminus (+) ( Figure 4E ), cytokeratin CAM5.2 (+, focal) ( Figure 4F ), AE1/AE3 (+, focal) (data not shown), EMA (-), calretinin (+, focal), SMARCB1/INI1(weak or loss) ( Figure 4G ).
case_images: MRI findings. Magnetic Resonance Imaging (MRI) revealed a light low signal compared to muscle on T1WI (A), a light high signal on T2WI with a capsule being low signal on T2WI (B, C) and a high signal on DWI (D). Delayed enhancement was observed on gadolinium-enhanced T1-weighted images (E). Cystic lesions inside of tumor (yellow asterisk) with high signals on T2WI (B) and without enhancement (E).
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case_images: DWI ( Figure 2D ). Delayed enhancement was observed on gadolinium-enhanced T1-weighted images ( Figure 2E ). In addition, there were cystic lesions inside the tumor with high signals on T2WI without enhancement, and a fluid level was observed inside the cystic lesion ( Figures 2B, E ).
case_images: Intraoperative and pathological findings of the resected specimen. the tumor (white line) had infiltrated the myocardium on left ventricle (yellow line) (A). Macroscopically, the tumor measured 12.5 x 9 x 3 cm with a grayish white color and bleeding (B). Ao; Aorta, PA; Pulmonary artery.
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case_images: The tumor was excised with careful avoidance of injury to the myocardium and coronary arteries, thus obviating the need for LV reconstruction ( Figure 3A ). Macroscopically, the tumor measured 12.5 x 9 x 3 cm and had a grayish white color with bleeding ( Figure 3B ).
case_images: Histopathological findings of cardiac monophasic synovial sarcoma. Resected specimens demonstrate that the tumor has spindle cell components with mitotic activity, focal necrosis, no rhabdoid cells (A-C). Immunohistochemical results for the indicated proteins are shown: (D) SS18::SSX; (E) SSX; (F) CAM5.2; (G) SMARCB1/INI1; (H) Ki67, magnification x400 (D, E), x200 (F-H). Fluorescence in situ hybridization showing SS18 split signal (I).
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case_images: Histopathologically, the tumor was composed of spindle cells, showing a monophasic pattern with mitotic activity (score 3), focal necrosis (score 1), no rhabdoid cells, and a staghorn-shaped vascular structure ( Figures 4A-C ). The results of immunohistochemistry (IHC) were as follows; SS18-SSX (-) ( Figure 4D ), SSX C-terminus (+) ( Figure 4E ), cytokeratin CAM5.2 (+, focal) ( Figure 4F ), AE1/AE3 (+, focal) (data not shown), EMA (-), calretinin (+, focal), SMARCB1/INI1(weak or loss) ( Figure 4G ). Figure 4H ). Fluorescence in situ hybridization (FISH) showed a SS18 split signal in 71 (71%) of 100 tumor cells ( Figure 4I ).