Management of liver tumors in childhood

Primary neoplasms of the liver occur rarely during childhood and constitute only 0.3-2% of all pediatric tumors. However, they comprise a variety of entities including benign and malignant epithelial, as well as mesenchymal tumors, the most common of these being hepatoblastoma and hepatocellular carcinoma. Clinical presentation, especially in young children is relatively uniform with abdominal enlargement and a painless tumor, and often specific symptoms develop late. Prerequisites for clinical diagnosis are a comprehensive laboratory workup and good quality imaging mainly with ultrasound, as well as CT and/or MRI scans. Histological diagnosis is essential for differential diagnosis and may only be omitted in some hepatoblastoma patients of the typical age (6 months to 3 years) with an excessively elevated serum-alpha-fetoprotein. Surgery is the mainstay of treatment for all benign and malignant liver tumors. Hepatoblastomas mostly respond well to chemotherapy. Therefore, this modality should always be combined with surgical resection in these patients and in many cases can reduce the size of a large tumor to resectability. Prognosis nowadays usually is good in all benign tumors and hepatoblastoma, as well as in some other rare malignancies, but dismal in hepatocellular carcinoma and other chemotherapy non-sensitive malignant tumors.