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Other considerations and issues in pediatric hepatology
Dolores López-Terrada, Baylor College of Medicine, Houston, TX, USA,
Milton J. Finegold, Department of Pathology and Immunology and Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA
OTHER CONDITIONS AND ISSUES IN PEDIATRIC HEPATOLOGY
Dolores López-Terrada, Assistant Professor, Department of Pathology, Baylor College of Medicine, Houston, Texas; Pathologist, Department of Pathology, Texas Children's Hospital, Houston, Texas,
Milton J. Finegold, Professor, Department of Pathology and Pediatrics, Baylor College of Medicine, Houston, Texas; Head of Pathology Department, Texas Children's Hospital, Houston, Texas
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).
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 . 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 . Primary liver tumors accounted for 6–8% of congenital tumors in Isaacs's review of 265 neoplasms discovered within 30 days of birth .