Management of liver tumors in childhood

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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.

Section snippets

Clinical presentation

Most children with a primary liver tumor present with abdominal distention and a palpable mass in the upper abdomen often without other signs of severe disease.2, 3 Anemia is often present. Only in advanced stage of disease, the good overall status deteriorates and the children develop abdominal pain, weight loss, nausea, vomiting, and ascites, and in case of pulmonary metastases progressive respiratory problems. Jaundice, signs of hepatic insufficiency or an incidental rupture of the tumor

Principles of tumor resection

The procedure of tumor resection in children has recently been thoroughly described by several authors.1, 2, 4 We choose a transverse incision combined with midline xiphoid extension if necessary. We mobilize the liver completely and put tourniquets around the hepatoduodenal ligament, as well as the vena cave above and below the liver for clamping if necessary. However, we try to avoid the Pringle maneuver and leave the remaining liver in the circulation during the resection to preserve optimal

Hepatoblastoma

From many studies, it is clear that there is only a chance for cure from HB if, at some time during the treatment procedure, a grossly complete resection of the tumor can be performed. Most HB, on the other hand, show good response to some cytotoxic drugs if given as neo-adjuvant chemotherapy reducing the tumor size and accomplishing better resectability. On this basis, two principle strategies exist. In the United States, a surgical tumor resection whenever possible is advocated as the first

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