Among pediatric patients with liver tumors, a review published in the Journal of Pediatric Gastroenterology and Nutrition described the most recent advances which are now available for these patients, including novel imaging techniques and locoregional therapeutic options.
The marker for liver tumors among children is primarily alpha-fetoprotein. However, not all tumors produce this marker. Liver tumors require imaging for diagnosis, such as ultrasound and magnetic resonance imaging (MRI).
Children can present with benign or malignant tumors. Among benign tumors there are 4 subtypes: hepatic hemangioma, mesenchymal hamartoma, focal nodular hyperplasia, and hepatic adenoma. Patients with these tumors present with abdominal distension and pain. The differentiation between subtypes is achieved via imaging. Treatment for benign tumors ranges from conservative management (focal nodular hyperplasia) to resection or liver transplant (mesenchymal hamartoma).
All children should be monitored after treatment to detect any changes to their liver tumor malignancy. Contrast-enhanced ultrasound is a visualization tool which does not require anesthesia and may be used in real-time, bed-side imaging. The use of this newer technology for children allows for the monitoring of benign liver tumors and has been incorporated into the European Association for the Study of the Liver guidelines.
Malignant lesions consist of 5 subtypes: hepatoblastoma, hepatocellular carcinoma, fibrolamellar carcinoma, metastatic liver lesions, and cholangiocarcinoma. Patients with these lesions often experience weight loss and may have hepatitis infections, glycogen storage disease, pepper syndrome, primary sclerosing cholangitis, or genetic syndromes such as Simpson-Golabi Behmel or Beckwith Weidman. Treatment for these tumors requires surgery and may require chemotherapy. Unlike those in adults ~50% of liver tumors among children are chemotherapy sensitive.
Malignant tumors are overall rare among children (~1%). Most malignant tumors have septa and/or pseudocapsule and are graded for risk on the basis of which hepatic veins they involve. Locoregional therapies are an option for patients with recurrent malignancies or tumors which are unresectable.
The review authors concluded that overall, liver tumors are rare among the pediatric population and that recent advances in diagnostic and personalized therapeutic options have improved patient care. Multiple worldwide trials are currently being conducted with the goal of better understanding clinical characteristics and treatment efficacy of liver tumors among children.
Kelgeri C, Renz D, McGuirk S, Schmid I, Sharif K, Baumann U. Liver tumours in children: the hepatologist’s view. J Pediatr Gastroenterol Nutr. 2021;72(4):487-493. doi: 10.1097/MPG.0000000000003006