Tumors of the liver may be either malignant or benign. The liver is the third-most-common site for intra-abdominal malignancy in children, following adrenal neuroblastoma and Wilms tumor. The incidence of primary malignant liver tumors per year is 1-1.5 per million children in the United States. This yields a relative low rate for hepatic tumors (1.3% of all pediatric malignancies). Of these malignant tumors, hepatoblastoma (HB) and hepatocellular carcinoma (HCC) are the most common and account for two thirds of all hepatic neoplasms. Benign liver tumors include hemangiomas, hamartomas, and focal nodular hyperplasia (FNH).
Presentation and workup
Most children with liver tumors present with abdominal distension, a palpable abdominal mass, or both. Anemia, thrombocytopenia, and leukocytosis are sometimes present. Children with both HB and HCC may also present with weight loss, fever, and anorexia.
Fetal and neonatal presentations include hydramnios, fetal hydrops, congestive heart failure, and respiratory distress. Patients with congestive heart failure have been shown to have lower survival rates. Cesarean delivery is recommended in cases when a hepatic tumor is found using prenatal ultrasonography to prevent rupture.
Laboratory studies are performed to assess baseline CBC count, electrolyte levels, liver enzyme levels, liver synthetic function, and α -fetoprotein (AFP) levels. AFP levels are elevated in 50%-70% of children with hepatic neoplasms, and multiple studies confirm that AFP is a valuable surveillance marker in children who have previously undergone hepatic resection for malignancy.
The initial workup for hepatic masses includes radiographic assessment using ultrasonography to confirm the location and to characterize the consistency as cystic or solid. Cystic or vascular lesions may not require any further imaging. CT scanning and MRI (MRI) of the abdomen and chest are used for indeterminate or solid lesions to further delineate the location, extent, and multiplicity of the lesions and to detect metastases. These modalities facilitate surgical planning and may determine resectability; however, definitive diagnosis can be proven only through biopsy findings.