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case_images: Intravenous bolus computed tomography of the abdomen showed a huge cystic mass with thick wall, occupying the most of the right lobe of the liver. The tumor wall has serpentine vascular enhancement during early contrast phase.
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case_images: Contrast enhanced abdominal computed tomographic (CT) scan showed a thick-walled cystic mass of 15 cm in diameter in the right hepatic lobe with serpentine enhancement in the wall during the early arterial contrast phase (Figure 1).
case_images: (A) Celiac angiography revealed a huge hypervascular mass in the right lobe of the liver. Tumor neovascularization was noted at the area of segments 4, 5, 6, 7 and 8. (B) Portovenography revealed total right portal vein defect.
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case_images: Angiography showed a hypervascular tumor which displaced hepatic artery branches in segments 5, 6, 7 and 8, and neovascularization was noted at the area of segments 4, 5, 6, 7, and 8 (Figure 2).
case_images: Gross examination of the excised tumor: Relatively well-demarcated, grayish-white mass measuring 7.5x16.0x4.5 cm in size showed expanding growth pattern. The tumor contained central cystic degeneration and homogeneous and homogenous solid portion.
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case_images: Several daughter nodules were noted (Figure 3).
case_images: Light microscopic examination : (A) Sections of the liver mass showed interlacing fascicles of spindle-shaped nuclei and perinuclear vacuoles (H&E, x200) (B) Tumor showed interlacing spindle-shaped cells arranged in fascicles, with hepatocyte present in the left side. (H&E, x200) (C) Tumor cells showed round nuclei with perinuclear vacuoles. The stroma revealed myxoid change. (H&E, x200)
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case_images: Most areas consisted of tumor cells which had typical cigar-shaped nuclei with a prominent nucleolus and fibrillary cytoplasm arranged in interlacing bundles and short fascicles (Figure 4). Occasionally, individual and/or aggregated primitive round tumor cells with vacuolated cytoplasm were scattered in the loose chondroid matrix (Figure 4).
case_images: Immunohistochemical stains of the resected mass showed positive staining for smooth muscle actin. (Immunohistochemical stain, x200)
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case_images: The tumor cells had spindle-shaped, hyperchromatic, occasionally pleomorphic nuclei with frequent mitotic figures (5-9/HPF). Immunohistochemical stains for vimentin, desmin, lysozyme and alpha1-antitrypsin were all positive, and keratin, actin and factor VIII were all negative (Figure 5).
case_images: Electromicroscopic examination showed myofilaments (arrow) with dense bodies. (EM, x6000)
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case_images: The tumor cells had spindle-shaped, hyperchromatic, occasionally pleomorphic nuclei with frequent mitotic figures (5-9/HPF). These findings confirmed the myofilamentous nature of the tumor (Figure 6).