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Rudolph's PediatricsRudolph's Pediatrics

Section 24. Neoplastic Disorders > 

Part 3. Solid Tumors > 

Chapter 458. Malignant Liver Tumors

Gail E. Tomlinson and Howard M. Katzenstein
Topics Discussed: beckwith-wiedemann syndrome; carcinoma, hepatocellular; familial adenomatous polyposis; hepatic neoplasm, childhood; hepatoblastoma; hepatocellular carcinoma, childhood; liver cancer, pediatric; liver cirrhosis; malignant neoplasm of liver; medical oncology; pediatric oncology.
Sections: References.
Excerpt:"Liver tumors represent approximately 1% of all childhood cancers. There are approximately 100 to 150 new cases of liver cancer diagnosed in children each year in the United States.1 Hepatoblastoma is the most common malignant tumor of the liver and accounts for two thirds of all liver cancer in children.2 Hepatoblastoma occurs almost always in the very young child, with a mean age of onset of approximately 18 to 20 months and 95% of cases occurring before 5 years of age. The incidence of hepatoblastoma has approximately doubled over the past few decades.3 Hepatoblastoma is considered an embryonal tumor because histologically, tumor cells resemble cells seen in the developing liver. Liver tumors in children of all ages most commonly present as an asymptomatic mass, although occasionally they are identified incidentally in patients with other symptoms, such as abdominal pain, nausea and vomiting, fever, and weight loss. Symptoms may sometimes indicate tumor rupture. The vast majority of children will have no evidence of underlying liver disease as jaundice, ascites, and abnormal liver function tests are usually not observed at presentation. Hepatoblastoma should be considered in patients with predisposing conditions such as a history of prematurity, hemihypertrophy, Beckwith-Weidemann syndrome, or a history of familial colon cancer. Hepatocellular carcinoma most commonly presents in an asymptomatic patient but sometimes arises in patients with a history of..."
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