Teratoid Hepatoblastoma with Multi-Lineage Differentiation: An Uncommon Histological Variant and Review of Literature

Background: Teratoid hepatoblastoma is an uncommon but well-recognized variant of mixed hepatoblastoma. Case report: A one-year female child presented with palpable and progressively increasing right abdominal mass for 3 months. The contrast-enhancing computed tomography (CECT) abdomen revealed a large heterogeneous hepatic mass measuring 12 x 6.6 x 6 cm. Histopathological examination of the resected specimen showed a mixed hepatoblastoma (epithelial and mesenchymal) with teratoid features and multi-lineage differentiation (all three germ cell layers). A focus showed embryonal rhabdomyosarcomatous element. Conclusion: Teratoid hepatoblastoma can show a wide range of heterologous differentiation that may pose a significant diagnostic dilemma. Such a broad spectrum has not been described in the literature previously. An appropriate immunohistochemical panel may be needed to identify and delineate the various heterologous differentiation to clinch the correct diagnosis. Secondary somatic malignancy such as rhabdomyosarcoma can develop in a teratomatous element. ( J CLIN EXP HEPATOL 2022;12:1393-1401)