PMC4058974_01
Initial abdominal computed tomography (CT) of diffuse hepatic hemangiomatosis and coexistent giant cavernous hemangioma in a 78-year-old man.. Axial contrast-enhanced CT image during the arterial phase shows heterogeneous nodular enhancement in both hemilivers and a contour-bulging mass with a central markedly low density area involving the caudate lobe. The expansile mass (arrowheads) protruded anteriorly, and the horizontal portion of the left portal vein was invisible, suggesting left portal vein occlusion by the mass effect of the caudate lobe lesion. The umbilical portion of the left portal vein was also invisible along the fissure for ligamentum teres (white arrow). Ascites in the right subphrenic space and dilated hepatic artery (long arrows) measuring 10-mm can be noted. There was no portomesenteric vein thrombosis (not shown).
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The CT demonstrated diffuse hepatic nodules involving the entire liver and a large heterogeneous mass replacing the left medial segment and caudate lobe (Fig. 1). It also showed peripheral punctate calcifications in the central hypodense area, suggesting phleboliths (Figs. 1, 2D). Furthermore, the horizontal and umbilical portions of the left portal vein could not be traced, suggesting left portal vein occlusion by central mass compression (Figs. 1, 2D).
Follow-up ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) in the same patient 2 months after symptoms had begun.. A, B. Abdominal US images show a large, heterogeneous hyperechoic mass with a central hypoechoic lesion (star) in the center of the liver, and a continuous hyperechoic area with a poorly defined margin of the mass (A). Numerous additional discrete and coalescent hyperechoic nodules (arrows), compared with normal parenchyma (asterisk), are scattered throughout the entire liver (B). C. US image in the hepatic hilum area shows hyperechoic bland thrombus (white arrows) along the main portal vein and dilated common hepatic artery (black arrows). D. Coronal contrast-enhanced CT image during the venous phase demonstrates heterogeneous nodular enhancement in both hemilivers. The large mass containing a central unenhanced, markedly hypodense area (star) replaces the medial segment with a bulging contour. There is portomesenteric bland thrombosis from the superior mesenteric vein to the main portal vein, part of the right portal vein (white arrows). Furthermore, the volume of the left lateral segment of the liver (asterisk) was decreased, suggesting hepatic lobar atrophy. It also shows calcification (black arrowheads) in the central large mass. The remaining ascites should be noted in both subphrenic spaces. E. T2-weighted MR image using half-Fourier single-shot turbo spin echo sequence (TR/TE infinite/103 ms, flip angle 107 ) demonstrates diffusely scattered nodular lesions with hyperintensity relative to the unaffected liver parenchyma (segment 7). This also shows marked hyperintensity of the central area (star). F, G. Serial gadolinium-enhanced T1-weighted MR axial images using a fat-suppressed, 3-dimensional volumetric interpolated breathhold examination sequence (TR/TE 3.5/1.5 ms, flip angle 9 ) show discontinuous nodular enhancement on the arterial phase (F) and gradual fill-in enhancing nodules turning more homogeneous but some remaining unfilled portions during the delayed phase (G), consistent with diffuse hepatic hemangiomatosis. The central markedly hypointense area (star) did not change without enhancement, corresponding to a degenerative change of the giant hemangioma. There was no distinct margin between giant hemangioma and adjacent diffuse hemangiomatosis. H, I. Histological findings reveal large, dilated vessels of varying size lined by flattened endothelium and arranged in a haphazard pattern (H; H&E, x200), and they are separated by fibrous septa of various thicknesses (I; Masson's trichrome stain, x100).
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On abdominal US, a large heterogeneous hyperechoic mass with a central hypoechoic portion was observed in the central hilus of the liver and the outer margin of the hyperechoic mass was not well defined (Fig. 2A). Numerous discrete and coalescent hyperechoic nodules, relative to the normal liver parenchyma, were scattered throughout the liver (Fig. 2B). In addition, hyperechoic bland portal vein thrombosis and dilated hepatic arteries were noted at the hepatic hilus level (Fig. 2C). It also showed peripheral punctate calcifications in the central hypodense area, suggesting phleboliths (Figs. 1, 2D). A central markedly hyperintense area was suspected to be a different histologic region, compared with the adjacent nodular lesions (Fig. 2E). Similar to the US images, there was no distinct margin between the giant hemangioma and the adjacent hemangiomatosis (Fig. 2F, G). Furthermore, the horizontal and umbilical portions of the left portal vein could not be traced, suggesting left portal vein occlusion by central mass compression (Figs. 1, 2D). Second, similar to the US findings, portomesenteric vein bland thrombosis was observed from the superior mesenteric vein to the main portal vein, and in part of the right portal vein (Fig. 2D). TSK Laboratory, Tochigi, Japan). The histological analysis revealed a typical cavernous hemangioma that had irregularly dilated nonanastomotic vascular spaces lined with flat endothelial cells alternating with normal hepatic parenchyma, without cellular atypia (Fig. 2H, I).
Previous abdominal ultrasonography of the same patient, taken 8 years before diagnosis of diffuse hepatic hemangiomatosis and coexistent giant cavernous hemangioma.. Diffuse and inhomogeneous echogenicity is located in the center of the liver.
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During a further workup, we obtained previous outside abdominal US images taken 8 years earlier, and they showed diffuse and inhomogeneous echogenicity throughout the liver, suggesting pre-existing diffuse hemangiomatosis (Fig. 3).