Teratoid hepatoblastoma with abundant cholangioblastic component in a child with full trisomy 13.

Teratoid hepatoblastoma is a rare histological subtype of hepatoblastoma. A 15-month-old girl with full trisomy 13 presented with a liver mass (6 × 4.4 × 3.4 cm). Histological examination showed a teratoid hepatoblastoma with very different differentiation patterns intermixed with each other. Approximately 30% of the tumor demonstrated a primitive glandular epithelium component, which had a moderate to well-differentiated adenocarcinoma-like morphology, and features of mucinous epithelium with a biliary immunophenotype. We designated it as cholangioblastic component. The child received 4 cycles of monotherapy with doxorubicin after complete resection and showed no evidence of residual tumor 8 months after surgery. Our case is not only the 1st report of hepatoblastoma in trisomy 13 but also represents a unique example with a large glandular epithelium component with cholangioblastic features. Increased awareness of this entity and further molecular studies are needed for better understanding of the pathogenesis of teratoid hepatoblastoma.