PMC4584445_01
31-year-old man with known glycogen storage disease Type-1a undergoing surveillance imaging. Dynamic multiphase contrast-enhanced MRI obtained in May 2014. (a) Pre-contrast T1W axial image shows two hepatic lesions in segments 4 (double arrows) and 8 (single arrow). (b) T1W axial image (arterial phase) shows variable heterogeneity foci of both of the lesions and arterial enhancement. (c) T1W axial image (venous phase) shows persistent enhancement of hepatic lesions. (d) T1W axial image (delayed or equilibrium phase) shows subtle washout in both of the lesions; posterior lesion also shows peripheral rim enhancement (double arrows).
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F1
The lesions showed variable heterogeneity and arterial enhancement with subtle washout [Figure 1].
31-year-old man with known glycogen storage disease Type-1a undergoing surveillance imaging diagnosed with hepatic adenoma. (a) T1W opposed-phase axial image shows diffuse loss of intensity of hepatic parenchyma (asterisk), which suggests the presence of diffuse hepatic steatosis. Two hepatic lesions in segments 4 and 8 are relatively hyperintense on the background of steatosis and lack microscopic fat. (b) T1W in-phase axial image shows two hepatic lesions in segments 4 (double arrows) and 8 (single arrow). (c) Fat-suppressed T2W axial image demonstrates isointense to slightly increased intensity of the above-described hepatic lesions (see the corresponding arrows), with an eccentric, ill-defined, T2-hyperintense possible scar.
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F2
T2-weighted (T2W) image, with an eccentric, ill-defined, T2-hyperintense possible scar, and did not reveal intralesional lipid. There was no T1 hyperintensity to suggest interval hemorrhage [Figure 2].
31-year-old man with known glycogen storage disease Type-1a undergoing surveillance imaging. Dynamic multiphase contrast-enhanced MRI obtained in Nov 2014 with gadolinium-EOB-DTPA. (a) Pre-contrast fat-suppressed T1W axial image shows two hepatic lesions in segments 4 (double arrows) and 8 (single arrow). The anterior lesion (single arrow) shows interval decrease in size, whereas the posterior lesion is slightly increased in size compared to MR dated May 2014 [Figure 2]. (b) Fat-suppressed T1W axial image (arterial phase) shows arterial enhancement of both hepatic lesions. (c) Fat-suppressed T1W axial image (venous phase) shows persistent enhancement of hepatic lesions; however, the anterior lesion (single arrow) appears fainter. (d) Fat-suppressed T1W axial image (delayed or equilibrium phase) shows remarkable washout in the posterior lesion (double arrows) with well-evident peripheral rim enhancement. (e and f) Fat-suppressed T1W axial image (hepatobiliary phases at 10 and 20 min, respectively) shows peripheral retention of the contrast in the anterior lesion (image f, star), which can be seen with inflammatory adenomas. The posterior lesion gradually becomes hypointense to the liver parenchyma and shows no uptake of contrast.
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F3
It was arterial enhanced with washout, with an enhancing rim on the venous phase, and did not show uptake of hepatocyte-specific contrast on the delayed phase images [Figure 3].
31-year-old man with known glycogen storage disease Type-1a undergoing surveillance imaging. Index images of two hepatic lesions with an interval of 6 months. (a) Fat-suppressed T1W fat post-contrast image acquired in Nov 2014. (b) Fat-suppressed T1W fat post-contrast image obtained in May 2014. The anterior lesion (single arrow) shows interval decrease in size on follow-up imaging, whereas the posterior lesion (double arrows) is slightly increased in size.
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F4
The size of the remaining adenomas either marginally decreased or was stable [Figure 4].
31-year-old man with known glycogen storage disease Type-1a. Post-biopsy histopathology of suspicious lesion seen on imaging. There is lack of portal tracts in all fragments. (a) In low-power magnification (hematoxylin and eosin stain), there is moderate steatosis (thin arrows) with many ballooned cells (thick arrow), some containing Mallory-Denk bodies (blue arrow). (b) In low-power magnification (hematoxylin and eosin stain), there are increased unpaired arteries (arrows). (c) In low-power magnification, there is focal Glypican-3 staining of the tumor cells. (d) In higher magnification, a reticulin stain shows focal loss of the reticulin framework (arrow).
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F5
A reticulin stain showed focal loss of the reticulin framework [Figure 5].