Malignant tumors originating in the liver are rare in children. They only account for 1.1% of pediatric malignant tumors.
Ninety percent of them are either hepatoblastomas (2/3) or hepatocellular carcinomas (1/3), the management, evolution, and prognosis of which are very different.
Malignant mesenchymal tumors account for the remaining 10%.
The most common sign of a liver tumor in children is usually swelling of the abdomen and, if the tumor is very large, pain or digestive problems.
Increased amounts of alpha-fetoprotein are used to diagnose hepatoblastoma or hepatocellular carcinoma and to monitor its progression under treatment.
Diagnosis also requires quality imaging (ultrasound, CT scan or MRI).
At the time of diagnosis, most children with hepatoblastoma are not showing any symptoms whereas in the case of hepatocellular carcinoma, there is often fever, pain, jaundice, weight loss and loss of appetite.
More rarely, the diagnosis will be made on account of precocious puberty related to the abnormal secretion of a hormone, beta human chorionic gonadotropin (or ß-HCG) by the tumor, irrespective of the tumor type: hepatoblastoma or hepatocellular carcinoma.
Most liver tumors are operated on after chemotherapy. Surgery should enable complete ablation of the tumor tissues and conserve the vessels needed for the irrigation and drainage of the remaining liver.
Surgical ablation of distant metastases is also part of the treatment given to children with hepatoblastoma when these metastases have not been controlled with chemotherapy. If the metastases are not widespread, resection of pulmonary metastases improves the prognosis. This can be performed at the same time as the liver tumor surgery.
Radiotherapy can have a place in the treatment of hepatoblastomas that have not been fully removed. However, its indications are very limited.
When the tumor has invaded the whole liver, a liver transplant may be indicated if the disease responded to initial chemotherapy. The prognosis of these children is the same as that of children who have had partial liver surgery: survival rate at 5 years reaches 80%.
In rare cases, hepatoblastomas that can be completely removed and with no metastasis can be treated with initial chemotherapy followed by post-operative chemotherapy.
The prognosis of hepatoblastoma is better than that of hepatocellular carcinoma.
Hepatocellular carcinoma is less sensitive to chemotherapy. Rare tumors operated on straightaway are treated post-operatively with a combination of Cisplatin and Doxorubicin. However, most children receive a preoperative chemotherapy regimen combining Cisplatinum, Doxorubicin and sometimes Carboplatin.
As with hepatoblastoma, if the tumor cannot be removed and it responds to initial chemotherapy, a liver transplant may be indicated. The prognosis of these children is the same as that of children who have had partial liver surgery. In children with hepatocellular carcinoma, local and/or metastatic relapses are more common. Response to treatment is approximately 50%. An exception to this rule is carcinoma known as fibrolamellar carcinoma which tends to progress slowly. 60% of these tumors are operable at diagnosis.