What is pediatric hepatoblastoma?
Hepatoblastoma is the most common type of liver cancer in children. It is a tumor which usually consists of embryonic or young liver cells.
What are the symptoms of hepatoblastoma in children?
Children who have hepatoblastoma are typically diagnosed when their parents or doctor notice a mass in the child’s abdomen. Sometimes the tumor is discovered when the patient complains of pain in the stomach or when a parent notices their child cradling the area or saying that their “tummy hurts”.
A yellowing of the skin and eyes, called jaundice, can also signal that a child is ill. This condition often signals that the liver may be involved.
In rare cases, boys may show signs of puberty at a very young age. Patients may be as young as 2-3 years old when the signs of early pubescence become apparent. This occurs when the tumor begins to secrete hormones, called beta-human choriogonadotropin hormone (β-HCG for short) , which trigger the body of male patients to start puberty at an abnormally young age. Like many other cancers, nausea and vomiting may also indicate the child is ill, but these symptoms may be so nonspecific that they are overlooked.
Who gets pediatric hepatoblastoma?
Hepatoblastoma is a very rare tumor. It occurs in approximately 1 to 2 children per million. In the United States there are about 100 cases per year.
Factors that may increase the risk of pediatric hepatoblastoma
- Having had a very low weight at birth,
- Having many members in the family who have had colon polyps or colon cancer (a condition known as familial adenomatous polyposis or FAP), or
- Having certain inherited condition such as Beckwith-Wiedemann Syndrome, trisomy 18, Simpson-Golabi-Behmel syndrome, Li-Fraumeni syndrome or Glycogen storage type I-IV.
How do you treat pediatric hepatoblastoma?
Surgery, chemotherapy drugs (cancer fighting medicines) and, when necessary, liver transplant are the main current treatments for hepatoblastoma. The particular treatment will be tailored to the patient and it will depend on whether the tumor has spread to organs other than the liver (this is called metastatic disease) and also on how difficult is to remove the tumor by surgery.
In most cases, the best treatment for hepatoblastoma is to remove the tumor. Unfortunately, only one-third to one-half of children who are newly diagnosed with hepatoblastoma have a tumor that can be removed by surgery at the time of presentation. If the tumor cannot be removed by surgery (called unresectable), chemotherapy can sometimes shrink the tumor. Once the tumor has shrunk, it may be possible to remove the tumor by surgery. If the tumor cannot be removed by surgery, either at diagnosis or after chemotherapy, the child may be evaluated for a liver transplant so that the entire liver and tumor can be removed and a new liver is put in to take the place of the old diseased one.
What doctors treat hepatoblastoma at Texas Children’s Cancer Center?
For a current list of oncologists and other physicians who treat hepatoblastoma at Texas Children’s Cancer Center, visit the Liver Tumor Program Members page.
Liver Tumor Imaging and Interventional Radiology
Most children with a liver tumor will have a doppler ultrasound as the initial imaging study. This type of imaging can distinguish solid masses from fluid-containing masses and determine whether or not the tumor contains lots of blood vessels. It can also show the relationship of the tumor to the normal blood vessels of the liver, and help target optimal sites for tumor biopsy. Magnetic resonance imaging (MRI) is the best known imaging study to provide precise anatomic detail, vascular information, and tumor size. This information is critical to determine whether the tumor is benign or malignant, whether it is resectable, if turns out to be a malignant cancerous tumor, how it responds to chemotherapy during treatment.. At Texas Children’s Hospital, we have created novel high-resolution MR sequences for measuring tumor volume, vascular anatomy, and perfusion, which can be performed with free-breathing without the need for endotracheal intubation and breath-holding.
Some liver tumors, such as vascular tumors like hemangioma, may benefit from targeted embolization. Targeted embolization is performed in a state-of-the-art interventional radiology laboratory inside Texas Children’s Hospital. The Interventional Radiology Laboratory is headed by physicians who are some of the foremost experts in the world in the study of hepatic vascular tumors.
Liver transplantation is required for children who have large liver tumors that cannot be surgically removed without causing liver failure. Liver transplants can be required for either benign or malignant tumors. The most common tumors that require liver transplantation are hepatoblastoma and hepatocellular carcinoma. However, transplants have been done for patients with sarcomas, adenomas, and even for large vascular malformations like hemangiomas.
The Texas Children’s Hospital (TCH) liver transplant programis the largest pediatric program in the South and among the largest in the United States. The program’s commitment to excellence is proven by its outcomes and the pediatric liver transplant survival rate at TCH is well above the national averages. Recent years, Texas Children’s Hospital performed 35-45 liver transplants on average per year.
Texas Children’s surgeons are experienced in a wide range of surgical procedures including liver resections, bile duct resections, and innovative liver transplantation techniques including living donor and in-situ split liver transplants. Split-liver transplants are procedures which the surgeon divides or splits the cadaveric liver while it is still in the donor’s body. This method allows the liver to be used for transplantation in two patients. Houston’s first in-situ split-liver transplant was performed in 1999 by Dr. John Goss. Living related donor transplantation is a technique in which a portion of an adult relative’s liver is removed and given to a child recipient. This type of transplantation virtually eliminates delays for a transplant because the child does not have to wait for a cadaveric liver transplant.