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Liver and Pancreas

  • Sarangarajan Ranganathan
Chapter
Part of the Molecular Pathology Library book series (MPLB)

Abstract

Hepatoblastoma (HB) is the most common malignant liver tumor of childhood. It is an embryonal tumor that has varied morphology, leading to its subclassification based on the histologic subtypes seen within the tumor. Molecular studies to date have consistently shown varying CTNNB1 (β-catenin) mutations or deletions in HB, and almost 90% of the tumors have some defect in the Wnt pathway. Varying partner pathways include the Notch pathway, EGFR pathway, YAP (HIPPO kinase pathway), c-myc, and possibly c-met and others that continue to be identified using more advanced molecular techniques. The author has tried to integrate this unique aspect of HB in which the morphology, the immunophenotype, and molecular events appear to go hand in hand. The chapter also looks at known prognostic markers as well as novel defects that seem to drive tumorigenesis in these patients.

Hepatocellular carcinoma (HCC) is the most common pediatric liver tumor in older children and adolescents. Rare instances of pediatric HCC occur in a background of normal liver, and activation of the Wnt/β-catenin pathway has been implicated in many of these pediatric tumors. Older children may develop the fibrolamellar variant of HCC that is unique in that it has a specific recently described translocation (DNAJB1-PRKACA). This chapter looks into the advances made in HCC in general, but with the hope of focusing more on the unique group of pediatric HCC tumors.

Pancreatoblastoma is an uncommon primitive tumor of childhood that shares in common with hepatoblastomas the presence of CTNNB1 mutations. The hallmark of the histologic diagnosis is the presence of squamous morular areas in a background of primitive tumor cells that may show acinic and neuroendocrine differentiation. While originally it was considered to represent the pediatric analog of the solid-pseudopapillary tumor due to its shared β-catenin staining, more recent data suggests that the adult counterpart may actually be an acinic cell carcinoma of the pancreas. The current chapter explores the scant molecular studies of these tumors.

Keywords

Hepatoblastoma Small cell undifferentiated β-catenin hepatocellular neoplasm NOS Hepatocellular carcinoma Hepatocellular adenoma Dysplastic nodule Fibrolamellar HCC Wnt/β-catenin pathway Pancreatoblastoma Acinic cell carcinoma 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of PathologyChildren’s Hospital of Pittsburgh of UPMCPittsburghUSA

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