Diffuse Adenomatosis and Hepatocellular Carcinoma Treated with Liver Transplantation in an Adolescent Female with Kabuki Syndrome with a Novel KMT2D Gene Mutation

Figure 1

(a) Axial T2 with fat saturation: large predominantly T2 hypointense mass in left hepatic lobe, T2 hyperintense scar internally, and heterogeneous liver parenchyma in the right lobe with suggestion of additional T2 hypointense masses. (b) Coronal postgadolinium LAVA: 19 min delay. Many of the masses retain hepatobiliary agent (Eovist) relative to the background liver on delayed series which suggests presence of functioning hepatocytes, typically seen in focal nodular hyperplasia, but can be seen in inflammatory subtype of adenomas and even well-differentiated HCC. Arrows designate multiple masses.

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