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Hepatoblastoma and the Hepatoblastoma Family of Tumors

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Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Abstract

Hepatoblastoma (HB) is a rare malignant blastomatous liver tumor characterized by various combination of epithelial and mesenchymal cell lineages that recapitulate early phases of liver ontogenesis. HB is typically a pediatric liver neoplasm; most cases are diagnosed before the age of 5 years. Very rarely, HB develops in older children and adults. HB accounts for less than 5 % of pediatric malignancies. The tumor occurs more commonly in low birth weight children and is associated with several molecular and cytogenetic abnormalities, mutations in the Wnt/beta-catenin signaling pathway being frequent. Morphologically, HBs are divided into epithelial and mixed epithelial-mesenchymal types. Epithelial tumors are broken down into fetal, embryonal, macrotrabecular, and small cell undifferentiated subtypes, while mixed epithelial-mesenchymal HBs are grouped into those without or with teratoid features. A subset of small cell undifferentiated HB exhibits rhabdoid features associated with loss of INI1 expression, similar to malignant rhabdoid tumors. Although HBs are aggressive lesions with high recurrence rate and metastatic disease, modern therapies now produce excellent results. In order to improve treatment, molecular profiles are developed to refine risk stratification.

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    Arthur Zimmermann

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Zimmermann, A. (2017). Hepatoblastoma and the Hepatoblastoma Family of Tumors. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_19

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