Hepatoblastoma

Condition: Hepatoblastoma (Liver Tumor)

Overview (“What is it?”)

  • Definition:  Hepatoblastoma is the most common liver tumor in children and usually is diagnosed as an abdominal mass or swelling in the belly that does not cause the child pain or discomfort. 
  • Epidemiology:  There are approximately 100 new cases of hepatoblastoma per year, or 1.6 children per million per year. A higher rate of this tumor is often found in low birth weight infants that are born prematurely. Since 1970s, the incidence of hepatoblastoma has nearly doubled for unknown reasons.
  • Hepatoblastoma tumors are mostly found in children six months to three years of age.

Diagnosis (“What tests are done to find out what my child has?”)

  • Labs and tests:
    • Blood Tests:  Several blood tests will be ordered if a liver tumor is suspected—routine labs to check for blood cell counts, liver enzyme amounts and some tumor markers such as alpha-fetoprotein (AFP).
    • Imaging studies:  Some or all of these studies may be performed:
      • Usually when there is belly swelling, plain abdominal X-rays are obtained first. X-rays are energy beams that go through the body onto a film, making a picture on the film.
      • Ultrasound uses sound waves to create images and pictures.
      • Computed tomography (CT) scans:  Detailed pictures of the chest or abdomen are taken, reconstructed in different views to get a better picture of the liver mass.  
      • MRI (Magnetic Resonance Imaging):  Uses a magnet, radiowaves and computer to obtain images of organs in the body. MRI does not use radiation. 
    • A biopsy or taking a piece of tissue from the mass, may be needed to make a definite diagnosis by looking at the tissue under a microscope.
  • Conditions that mimic this condition: Other non-cancerous liver tumors/masses such as benign hemangiomas (blood vessel abnormality) or hamartomas can mimic this tumor. Other cancerous masses that arise from the liver are hepatocellular carcinoma, germ cell tumors (infantile choriocarcinoma of the liver), undifferentiated embryonal sarcoma of the liver. 
  • There are several congenital (baby is already born with) conditions that are associated with hepatoblastoma such as Beckwith-Wiedemann syndrome, Trisomy 18, low birth weight infants and Familial Polyposis syndromes, to name a few.

Treatment (“What will be done to make my child better?”)

  • Staging:  In all types of cancer, it is important to determine if the cancer is isolated or has spread through the body. The treatment is dependent on the stage of the cancer.
    • In hepatoblastoma, staging is done to look at whether the tumor can be completely removed from the liver and still leave enough liver to have function. Staging will also be done to see whether there is cancer spread in organs other than the liver.
    • A pediatric oncologist (doctor that specializes in treating pediatric cancer) will guide you through the types of medicines and radiation to be used.
  • Medicine:  Chemotherapy are drugs that are especially aimed at destroying hepatoblastoma cells, are used either before AND after surgery or just after surgery. These are given through the vein.
  • Radiation therapy may also be used if any tumor cells are left behind after surgical removal of the mass.
  • Surgery:  Due to the rare and complex nature of these tumors, treatment should be performed at centers/hospitals where surgeons are very familiar with hepatoblastoma. Tumors are removed if they are able to be completely resected and still leave the patient with enough liver remaining to support the patient. Occasionally, liver transplant surgeons will be involved in the decision making process as well, because transplant is sometimes the best option in certain situations.
  • The mass/tumor can usually be resected at the time of diagnosis in about one-third of patients. The other two-thirds have tumors that are too large to be removed right away and will get chemotherapy first. The remaining liver will regrow and function normally. 
    • Preoperative preparation:  Your child will require general anesthesia for any surgical procedure so he/she will have to stop eating several hours before the surgery. A shower or a bath the night prior or the day of surgery helps cleanse the skin to decrease wound infections. Certain labs may be drawn to check blood count levels and to check the function of the liver.
    • Postoperative care:  Your child will likely remain in the hospital for several days following the surgery in order to provide good pain control and intravenous fluids. Once he/she is eating well and able to take medications by mouth, they will be discharged.
    • Central Line Placement (Port-A-Cath or Broviac Catheter) will likely be necessary to give chemotherapy drugs before or after removing the liver tumor.
    • Metastases (pieces of tumor that have spread to other parts of the body, usually the lung) may require removal by surgery as well.
  • Risks/Benefits:
    • The main risks of surgery are bleeding and infection. Your child will likely have their blood type checked before surgery in case a blood transfusion will be necessary. They will also be given antibiotics before and maybe after surgery to help reduce the chance of infection. 
    • The other risk of surgery is not getting all the tumor out. This may mean that your child will need additional surgeries in the future or additional chemotherapy to rid the liver of cancer cells.
    • There are risks to the drugs used to treat the tumor as well and include heart and kidney problems, lowering of blood cells, developing other tumors and the risk of infection (usually from the central line).

Home Care (“What do I need to do once my child goes home?”)

  • Diet:  Your child will likely be able to resume a normal diet without restrictions.
  • Activity:  Depending on the extent of surgery, your child might need to take it easy for a few weeks after surgery. Children tend to recover faster than adults, so they may be able to return to school and light-duty activities within a week or two.
  • Wound care:  Your surgeon should inform you of any specific wound care and whether or not you can get the incision wet. Call your surgeon if there is any redness or drainage from the incision or if your child has any fevers. You will also be given instructions in how to care for the central line.
  • Medicines:  You may be given a prescription for pain medications. Depending on the tumor, your child may need to return to the hospital or clinic to receive chemotherapy (drugs that attack cancer cells).
    • In patients with cancer, especially those who are on chemotherapy, acetaminophen (Tylenol®) or ibuprofen (Motrin® or Advil®) should be avoided as fevers due to infection may be masked.
  • What to call the doctor for:  Call your surgeon for fevers (greater than 101° Fahrenheit), redness or drainage from the incision or for any vomiting or diarrhea.
  • Follow up care:  You will generally need to see your surgeon one to two weeks after your surgery and will also have a follow up with your oncology doctor.

Long-Term Outcomes (“Are there future conditions to worry about?”)

  • The overall survival of babies with hepatoblastoma has been steadily improving over the last few decades. The prognosis or chance that your child will do well is dependent on success of removing the tumor and how well he/she responds to the medications.
  • Your child will require long-term follow up with the oncologist as well as the surgeon to monitor for tumor coming back and for possible side effects of treating the tumor. This may involve getting your child's blood checked periodically as well as having imaging studies performed such as CT scans or MRIs.  
  • If the tumor cannot be completely removed or returns after treatment, then a liver transplant may be necessary, and follow-up with a transplant surgery team may be arranged.

References:

  1. Pediatric Surgery; Coran, Arnold G.  Copyright © 2012, 2006 by Saunders, an imprint of Elsevier Inc.
  2. National Cancer Institute  http://www.cancer.gov/types/liver/hp/child-liver-treatment-pdq#link/_568_toc.


Updated: 11/2016
Author: Patricia Lange, MD
Editor: Marjorie J. Arca, MD