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40 - Tumors of the Liver

from SECTION V - OTHER CONDITIONS AND ISSUES IN PEDIATRIC HEPATOLOGY

Published online by Cambridge University Press:  18 December 2009

By
Dolores López-Terrada M.D., Ph.D.  and
Milton J. Finegold M.D.
Edited by
Frederick J. Suchy ,
Ronald J. Sokol  and
William F. Balistreri
Dolores López-Terrada M.D., Ph.D.
Affiliation:
Assistant Professor, Department of Pathology, Baylor College of Medicine, Houston, Texas; Pathologist, Department of Pathology, Texas Children's Hospital, Houston, Texas
Milton J. Finegold M.D.
Affiliation:
Professor, Department of Pathology and Pediatrics, Baylor College of Medicine, Houston, Texas; Head of Pathology Department, Texas Children's Hospital, Houston, Texas
Frederick J. Suchy
Affiliation:
Mount Sinai School of Medicine, New York
Ronald J. Sokol
Affiliation:
University of Colorado, Denver
William F. Balistreri
Affiliation:
University of Cincinnati
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Summary

The clinical presentation of the vast majority of liver tumors in children is an asymptomatic palpable mass. The majority of malignancies are large and may be difficult to excise without prior chemotherapy because the liver's functional capacity is rarely compromised by underlying cirrhosis. Involvement of the perihilar segments or intrahepatic dissemination may necessitate transplantation. The vascularity of the liver and ready access of cancer cells to hepatic veins make pulmonary metastasis at presentation relatively common. Therefore, knowledge of precursor conditions and screening can be lifesaving.

Approximately two thirds of all liver masses occurring in children are malignant. Twenty separate series totaling 1972 primary benign and malignant liver tumors in children from 1956 to 2001 included hepatoblastomas (HB; 37%), hepatocellular carcinomas (HCC; 21%), benign vascular tumors (15%), mesenchymal hamartomas and sarcomas (8%), adenomas and focal nodular hyperplasia (7.5%), and other tumors (4%) [1, 2] (Table 40.1).

EPIDEMIOLOGY

Approximately 1.1% of all childhood tumors in the United States are malignant liver tumors according to the Surveillance, Epidemiology, and End Results (SEER) program cancer registries, with an annual incidence rate of 1.8 cases per million children younger than 15 years [3]. Of 123 children registered with malignant liver tumors in 2000, 80% had HB and they accounted for 91% of primary hepatic malignancy cases in children aged less than 5 years [4]. Primary liver tumors accounted for 6–8% of congenital tumors in Isaacs's review of 265 neoplasms discovered within 30 days of birth [5].

Type
Chapter
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Liver Disease in Children , pp. 943 - 974
Publisher: Cambridge University Press
Print publication year: 2007

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  • Tumors of the Liver
    • By Dolores López-Terrada, M.D., Ph.D., Assistant Professor, Department of Pathology, Baylor College of Medicine, Houston, Texas; Pathologist, Department of Pathology, Texas Children's Hospital, Houston, Texas, Milton J. Finegold, M.D., Professor, Department of Pathology and Pediatrics, Baylor College of Medicine, Houston, Texas; Head of Pathology Department, Texas Children's Hospital, Houston, Texas
  • Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
  • Book: Liver Disease in Children
  • Online publication: 18 December 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511547409.042
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  • Tumors of the Liver
    • By Dolores López-Terrada, M.D., Ph.D., Assistant Professor, Department of Pathology, Baylor College of Medicine, Houston, Texas; Pathologist, Department of Pathology, Texas Children's Hospital, Houston, Texas, Milton J. Finegold, M.D., Professor, Department of Pathology and Pediatrics, Baylor College of Medicine, Houston, Texas; Head of Pathology Department, Texas Children's Hospital, Houston, Texas
  • Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
  • Book: Liver Disease in Children
  • Online publication: 18 December 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511547409.042
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  • Tumors of the Liver
    • By Dolores López-Terrada, M.D., Ph.D., Assistant Professor, Department of Pathology, Baylor College of Medicine, Houston, Texas; Pathologist, Department of Pathology, Texas Children's Hospital, Houston, Texas, Milton J. Finegold, M.D., Professor, Department of Pathology and Pediatrics, Baylor College of Medicine, Houston, Texas; Head of Pathology Department, Texas Children's Hospital, Houston, Texas
  • Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
  • Book: Liver Disease in Children
  • Online publication: 18 December 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511547409.042
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