case_images: PMC6142750_01
case_images: (a) Ultrasonography of the liver. Grayscale with Doppler image demonstrates a partially defined hepatic mass with multiple internal cystic foci (arrowheads) and increased intralesional vascularization (arrows). MRI of the abdomen with axial T2 fat saturation (b) and LAVA (liver acquisition with volume acceleration sequence) after intravenous contrast administration at 5 min (c) and 20 min (d) show a large heterogeneous mass within the right hepatic lobe. A predominant intralesional cystic/necrotic component (*), multiple internal septations (arrows), and residual solid tissue (arrowheads) are seen.
case_images: CRIONM2018-7840865.001.jpg
case_images: PMC6142750_01_CRIONM2018-7840865.001.jpg
case_images: fig1
case_images: Ultrasonography revealed a partially defined hepatic mass with multiple internal cystic foci, and an increased intralesional vascularization is identified (Figure 1(a)). Magnetic resonance imaging (MRI) of the abdomen revealed a 17 x 18 x 20 cm heterogeneous predominantly cystic mass with thick internal septations, residual solid tissue, and peripheral neovascular formation in the right hepatic lobe (Figures 1(b)-1(d)).
case_images: Pathological findings of the UESL. (a) Macroscopic appearance of the tumor. Histological examination showed (b, c, and d) hypocellular myxoid and hypercellular areas containing stellate and spindle-shaped malignant cells with scattered bizarre multinucleated giant cells, (e) the presence of cytoplasmic eosinophilic hyaline globules (arrow), and (f) cords of hepatocytes and bile ducts entrapped within the tumor (hematoxylin and eosin stain; original magnification: (b) x100; (c, d, and f) x200; (e) x400).
case_images: CRIONM2018-7840865.002.jpg
case_images: PMC6142750_01_CRIONM2018-7840865.002.jpg
case_images: fig2
case_images: Cut surface of the tumor showed a variegated appearance of gray, solid glistening tumor alternating with soft gelatinous areas with dark-brown and yellow-green areas of hemorrhage and necrosis (Figure 2(a)). It was comprised predominantly of pleomorphic cells that are spindle, oval, or stellate shaped and distributed in a fibrous or myxoid stroma (Figures 2(b)-2(d)). Few sharply defined eosinophilic hyaline globules in the tumor cell cytoplasm were observed (Figure 2(e)). Entrapped bile ducts and hepatic cords were present in areas at the periphery of the tumor (Figure 2(f)).
case_images: Immunohistochemical staining of the tumor cells in the UESL showed strong positivity for vimentin (a) and alpha1-antitrypsin (b), patchy positivity for desmin (c), and negative for myogenin (d), smooth muscle actin (SMA) (e), and pancytokeratin AE1/AE3 (f). The entrapped hepatic cords were negative for AE1/AE3 (f) (arrowhead), but AE1/AE3 highlighted the entrapped bile ducts (f) (arrow) (immunoperoxidase; original magnification: x200).
case_images: CRIONM2018-7840865.003.jpg
case_images: PMC6142750_01_CRIONM2018-7840865.003.jpg
case_images: fig3
case_images: By immunohistochemistry, tumor cells stained positively for vimentin and alpha1-antitrypsin, partially positive for desmin, and negative for myogenin, smooth muscle actin (SMA), and pancytokeratin (AE1/AE3) (Figures 3(a)-3(f)). The AE1/AE3 stain highlighted the entrapped bile ducts (Figure 3(f)).
case_images: Pulmonary metastatic lesion of UESL. (a) CT of the thorax showed a well-defined solid nodular lesion (arrow) in the left lower lobe. (b, c) Histological examination of the lung lesion. (d) The uninvolved pulmonary parenchyma (to upper right of the center) is compressed. (e, f) Bronchioles entrapped within and at the periphery of the tumor. An isolated bronchiole is surrounded and focally destroyed by the infiltrating tumor (f) (arrow) (hematoxylin and eosin stain; original magnification: (f) x40; (d) and (e) x100; (b) and (c) x200).
case_images: CRIONM2018-7840865.004.jpg
case_images: PMC6142750_01_CRIONM2018-7840865.004.jpg
case_images: fig4
case_images: Chest CT imaging at 50 months following the hepatectomy revealed a 1.7 x 1.4 cm lung nodule in the left lower lobe (Figure 4(a)) with no pleural or pericardial effusions. Histopathological studies revealed that the tumor was composed of pleomorphic stellate and spindled neoplastic cells in a predominantly myxoid matrix; scattered bizarre multinucleated giant cells and atypical mitotic figures were frequently seen (Figures 4(b)-4(d)). There were bronchioles entrapped within and at the periphery of the tumor (Figures 4(e) and 4(f)).
case_images: Immunohistochemical staining of the pulmonary metastatic lesion showed strong and diffuse positivity for vimentin, alpha1-antitrypsin, and CD10 (a-c), patchy positivity for desmin, CD56, and BCL2 (d-f), rare staining for CD68 (g), and negative for myogenin and AE1/AE3 (h and i). The entrapped bronchiolar epithelium was highlighted by AE1/AE3 (i) (arrow) (immunoperoxidase; original magnification: (c, d, g, and i) x100; (a, b, e, f, and h) x200).
case_images: CRIONM2018-7840865.005.jpg
case_images: PMC6142750_01_CRIONM2018-7840865.005.jpg
case_images: fig5
case_images: By immunohistochemistry, the tumor cells were strongly and diffusely positive for vimentin, alpha1-antitrypsin, and CD10, patchy positive for desmin, CD56, and BCL2, rare staining for CD68, and negative for myogenin and AE1/AE3 (Figures 5(a)-5(i)). The entrapped bronchiolar epithelium was highlighted by AE1/AE3 (Figure 5(i)).