Beta cateninc adenomas and hepatoblastoma in familial adenom | Figure 1

CincyKidsRad

Cincinnati Children's Radiology

Beta cateninc adenomas and hepatoblastoma in familial adenomatous polyposis

Axial T1-weighted postcontrast imaging in the hepatobiliary phase (image 1), T2-weighted imaging (image 2), T1-weighted imaging in the arterial phase (image 3), and axial contrast-enhanced CT (image 4) display a hepatoblastoma (arrow) in the left hepatic lobe. Multiple hepatobiliary phase hyperenhancing lesions (arrowhead) within the visible right liver are present. These lesions are not visible on other phases of imaging. This finding is characteristic of beta-catenin-activated hepatocellular adenomas in patients with familial adenomatous polyposis.

More about this case

Resolved - Rare - Trending

Teaching Points:

  1. Hepatoblastoma: Most common hepatic malignancy of childhood. It is associated with prematurity and syndromes such as Beckwith-Wiedemann syndrome or familial adenomatous polyposis.
  2. Imaging Characteristics: Staged using the PRETEXT system.

Images and Descriptions:

Axial T2 weighted (image 1), coronal T2 weighted (image 2), axial T1 post contrast in the portal venous phase of imaging (image 3) and coronal postcontrast in the hepatobiliary phase of imaging (image 4) show the large mass (arrow) of the right hepatic lobe. In addition, there is thrombosis of the inferior vena cava (arrowhead) and portal vein (more posterior arrow on image 3).

Axial T2-weighted (image 1), T1-weighted (image 2), and postcontrast hepatobiliary phase (image 3) MR images show a poorly defined area of signal abnormality (arrow) within the central portion of the liver, representing nodular regenerative hyperplasia. This pattern is common in patients with Alagille syndrome and other cholangiopathic disorders.

Axial T2-weighted (image 1) and T1-weighted postcontrast MRI in the hepatic arterial (image 2) and hepatobiliary phases (image 3) show a large central hepatic mass (arrow) with a heterogeneous appearance. The internal scar observed is hypointense on the hepatobiliary phase and not visible on the portal venous phase.

Axial T1-weighted, T2-weighted, and opposed-phase MRI of the spleen shows two splenic hamartomas (arrow). Splenic hamartomas are rare tumors associated with tuberous sclerosis, displaying heterogeneous appearance due to foci of hemorrhage.