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Hepatic Tumors in Childhood

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Pathology of Pediatric Gastrointestinal and Liver Disease

Abstract

Great progress in the treatment of hepatoblastoma and some other tumors of the liver in children has been achieved in the past quarter century. Mostly, the benefits have derived from cis-platinum-based chemotherapy for neoplasms and propranolol for infantile hemangiomas. With the advances has come the recognition that thorough histopathologic characterization of HB has prognostic significance and should influence the choice of therapy. These advances have done less for disease that is all too often disseminated at presentation, including most hepatocellular carcinomas and the small undifferentiated cell and rhabdoid variants of HB. The rarity of the tumors has led to increasing international collaboration among all disciplines to address the outstanding issues. Molecular genetic research on pathways of gene expression is providing insights into carcinogenesis and will lead to therapies that avoid the toxicity of current regimens while more effectively attacking the neoplastic cells. But traditional morphologic diagnoses and complete surgical extirpation, including transplantation, remain essential. This chapter summarizes the current data on the antecedent and acquired genetic abnormalities in liver tumors while emphasizing those aspects of histopathology that are essential for diagnostic accuracy and prognosis.

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Acknowledgments

The authors are grateful to Christie Hollins for preparing the manuscript, including the complex tables, and Karen Prince and Dr. Rishi Chavan with crucial help with the digital images. Colleagues in the Children’s Oncology Group and worldwide provided clinical data and tissue samples that informed and stimulated the authors.

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  1. Department of Pathology & Immunology, Baylor College of Medicine, One Baylor Plaza, Houston, TX, 77030, USA

    Milton J. Finegold MD & Dolores H. López-Terrada MD, PhD

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  1. Milton J. Finegold MD
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  2. Dolores H. López-Terrada MD, PhD
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Correspondence to Milton J. Finegold MD .

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Editors and Affiliations

  1. Department of Pathology, Division of Anatomic Pathology, The Children’s Hospital of Philadelphia, and the Perelman School of Medicine at The University of Pennsylvania, Philadelphia, Pennsylvania, USA

    Pierre Russo

  2. Department of Pathology, Division of Anatomic Pathology, The Children’s Hospital of Philadelphia, and the Perelman School of Medicine at The University of Pennsylvania, Philadelphia, Pennsylvania, USA

    Eduardo D. Ruchelli

  3. Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, The Children’s Hospital of Philadelphia, and the Perelman School of Medicine at The University of Pennsylvania, Philadelphia, Pennsylvania, USA

    David A. Piccoli

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Finegold, M.J., López-Terrada, D.H. (2014). Hepatic Tumors in Childhood. In: Russo, P., Ruchelli, E., Piccoli, D. (eds) Pathology of Pediatric Gastrointestinal and Liver Disease. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-54053-0_14

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