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Hepatoblastoma: New Insights into the Biology of Embryonal Tumors of the Liver

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Pediatric Neoplasia

Part of the book series: Molecular and Translational Medicine ((MOLEMED))

Abstract

Malignant tumors of the liver are rare in children, with an approximate incidence of 1.8 cases per million, representing 1 % of all malignancies diagnosed during childhood. Hepatocellular neoplasms are the most common type of primary tumors of the liver in children, with hepatoblastoma (HB) being the most common during the first years of life (median age of diagnosis is 1 year), followed by hepatocellular carcinoma, usually affecting older children and adolescents (Hepatology 38:560–566, 2003; Hum Pathol 14:512–537, 1983). Several sources have reported an increase in the incidence of HB during the last two decades, both in the United States and in Japan, as well as a significantly higher rate of HB among low and very low birth weight infants. Primary liver tumors account for 6–8 % of all congenital tumors. HB has been associated with a number of constitutional genetic abnormalities, malformations, familial cancer syndromes (familial adenomatous polyposis, Beckwith–Wiedemann syndrome), and metabolic disorders (Glycogen storage diseases types I and IV) (Liver Disease in Children, 2007). However, most HBs are sporadic and the etiology for these tumors is not presently known.

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Acknowledgment

The author would like to sincerely thank Kayuri Patel and Karen Prince for their valuable assistance in the creation of the figures and editing of this chapter.

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López-Terrada, D. (2012). Hepatoblastoma: New Insights into the Biology of Embryonal Tumors of the Liver. In: Mackinnon Jr, A. (eds) Pediatric Neoplasia. Molecular and Translational Medicine. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-116-5_11

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