What Is a Rhabdoid Tumor of the Liver?

A rhabdoid (RAB-doyd) tumor of the liver is a cancer that begins in the liver, then often spreads quickly to other parts of the body. These tumors are very rare and usually happen in babies and toddlers. Rhabdoid tumors also can affect other parts of the body, like the kidneys and brain.

What Are the Signs & Symptoms of a Rhabdoid Tumor of the Liver?

A child with a rhabdoid tumor might have:

• a large belly that sticks out
• belly pain, most often on the right side
• belly mass (something solid in the belly that can be felt through the skin)
• jaundice (yellow skin or eyes)
• back pain
• a fever
• itching
• loss of appetite
• weight loss
• nausea and vomiting
• anemia (low red blood cell count)

What Causes a Rhabdoid Tumor of the Liver?

Healthy cells have a way of making sure that they grow as they should. If something goes wrong in the cells, they can grow out of control. Most of the cells that grow out of control and form a rhabdoid tumor of the liver have lost at least a piece of a normal gene (SMARCB1 or INI1).

Rhabdoid tumors of the liver usually develop in the first year of life. The average age at diagnosis is 15 months.

How Is a Rhabdoid Tumor of the Liver Diagnosed?

A doctor who is concerned that a child has a rhabdoid tumor of the liver will do an exam and order tests. These may include:

• blood tests, including liver and kidney function tests and an alpha fetoprotein (AFP) test (liver damage and some cancers can raise the level of this protein in the blood)
• imaging tests:
  • ultrasound
  • X-rays
  • CAT scan
• a biopsy: removing a piece of tumor tissue for examination or testing

How Is a Rhabdoid Tumor of the Liver Treated?

Doctors usually treat rhabdoid tumors of the liver with a combination of surgery and  chemotherapy. If possible, children with cancer should go to a medical center specializing in the treatment of pediatric cancers.

Treatment depends on:

• the child's age
• the size of the tumor
• whether there is one or many tumors in the liver
• whether the cancer has spread from the liver

Treatment Options

Surgery is needed to cure rhabdoid tumor of the liver. But because of the size of the tumor, it's not possible in most children at the time they are first diagnosed.

Chemotherapyis often used, but typically is only partially effective in shrinking the rhabdoid tumor.

Radioembolization (or Y90) is a type of therapy that delivers high-dose radiation directly to the liver tumor through the bloodstream. The radiologist inserts a tiny catheter (plastic tube) in the groin and passes it to the artery closest to the tumor. This procedure protects much of the normal liver tissue from the effects of Y90.

Y90 can be used as primary therapy in liver tumors that don't respond well to chemotherapy. It's also used when the tumors come back or don't shrink enough for surgery.

Transarterial chemoembolization (TACE), which sends chemotherapy particles directly to the tumor.

Tumor ablation, which is when doctors destroy tumors by using small needles to heat or cool them.

Sometimes, the entire liver must be removed to treat the cancer. The liver is a vital organ, so in these cases a child will need a liver transplant.

Who Treats Rhabdoid Tumor of the Liver?

Rhabdoid tumor of the liver is treated by a health care team, including specialists in:

• oncology (cancer)/hematology (blood diseases)
• surgery
• interventional radiology (image-guided minimally invasive procedures)
• gastroenterology (digestive tract) and hepatology (liver)
• pathology (diagnosing diseases by examining body tissues, fluids and organs)
• genetics (genetic counseling and testing)
• radiology (medical imaging)
• nutrition

What Else Should I Know?

Research into better treatments for rhabdoid tumors is ongoing. If your child is being treated for a rhabdoid tumor of the liver, you don't have to go it alone. To find support, talk to your child's doctor or a hospital social worker. Many resources are available to help you get through this difficult time.

You also can find information and support online at:

• National Cancer Institute
• American Childhood Cancer Organization
• American Liver Foundation