Hepatoblastoma

  • Etiology: Tumor of embryonic origin
  • Imaging: Staged via PRETEXT system with need to characterize the lesion and assess for surgical resectability:
    — Tumor can be focal or multifocal
    — Can have hemorrhage and necrosis
    — Local or regional spread to lymph nodes and adjacent structures
    — Distant spread to lungs
    — Vascular invasion
  • Imaging CT:
    — Has coarse or chunky calcification
    — Enhances irregularly and less than the liver
  • DDX: Hepatic hemangioma
  • Complications: Metastases to lung (17%) and lymph nodes,
    vascular obliteration and vascular encasement and vascular invasion, tumor thrombus,
    peritoneal nodules, rupture, multifocality, patients with newly diagnosed hepatoblastoma have high incidence of fractures
  • Treatment: Chemotherapy then surgery
  • Clinical:
    — Elevated AFP (90%)
    — Premature infants are at increased risk
    — 70% present in first 2 years and 90% present by 5 years
    — 4% congenital
    — Associated with Beckwith-Wiedemann syndrome and Gardener syndrome and familial adenomatous polyposis and Trisomy 18 and Type 1A glycogen storage disease

Radiology Cases of Hepatoblastoma

CT and US and angiogram of hepatoblastoma
Axial CT without (above left) and with (above right) contrast of the abdomen shows a large, coarsely calcified, heterogenously enhancing liver mass. US image (below left) shows its multinodular nature and an angiogram (below right) shows its neovascularity.
CT of hepatoblastoma
Axial (above), coronal (below left) and sagittal (below right) CT with contrast of the abdomen shows a large mass in the right lobe of the liver with punctate calcifications that is heterogenous in appearance and with a low density center, encases the portal veins, and enhances less than the liver.
CT and MRI of hepatoblastoma
Axial CT with contrast of the abdomen (above left) shows a large, inhomogenous multifocal mass on the right side of the abdomen that enhances less than the liver and that did not appear to arise from the right adrenal gland or right kidney. Axial T2 (above right) and axial (below left) and coronal (below right) T1 MRI with contrast of the abdomen show the mass arising from and involving nearly the entire liver and encasing the portal veins.