Rhabdoid Tumor of the Liver
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Rhabdoid Tumor of the Liver

What Is a Rhabdoid Tumor of the Liver?

A rhabdoid tumor of the liver is a cancer that begins in the liver, then often spreads quickly to other parts of the body. Most of these very rare tumors happen in babies and toddlers.

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

A rhabdoid (RAB-doyd) tumor of the liver doesn't usually cause symptoms until it spreads to other parts of the body. In its early stages, the tumor may cause symptoms of a viral infection. So a child might:

  • have a fever
  • not eat as much as usual
  • vomit (throw up)
  • not play or move around as much as usual

As the tumor grows, a child's symptoms might include:

  • a belly that looks uneven
  • a belly that looks or feels larger than usual
  • a hard growth (called a mass) that parents or doctors can feel in the child's belly
  • trouble breathing, if the tumor has spread to the lungs

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 one piece of DNA (gene).

Who Gets a Rhabdoid Tumor of the Liver?

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

Some families are more at risk for rhabdoid tumors because of a change in their DNA that passes from one generation to the next.

How Is a Rhabdoid Tumor of the Liver Diagnosed?

Other conditions can cause similar symptoms, so doctors work step-by step to diagnose a rhabdoid tumor of the liver.

First, the doctor will do an exam and treat the child for the most common causes of the symptoms. To look for a more serious problem, the doctor will order blood tests and one or both of these imaging studies:

These studies can detect a liver tumor and show whether it has spread (metastasized) to other parts of the body. The doctor will send the child to a cancer specialist who will work with a team of specialists to find out what kind of tumor it is and decide how best to treat it.

One of the early steps in making the diagnosis is doing a biopsy. Using a needle, the doctor will take a sample of the tumor. The sample is checked in a lab to see if the cells look like those of a rhabdoid tumor.

How Is a Rhabdoid Tumor of the Liver Treated?

Doctors usually treat rhabdoid tumors of the liver with a combination of surgery, chemotherapy, and radiation. But they are hard to treat successfully.

The treatment team will include pediatric experts in:

  • surgery
  • hematology/oncology (blood diseases and cancer)
  • pathology (diagnosing diseases by examining body tissues, fluids, and organs)
  • radiology (medical imaging)
  • interventional radiology (image-guided minimally invasive procedures)

The team reviews all available research about the best ways to treat the tumor. Options can include:

Surgery

A pediatric surgeon removes the tumor along with a part of the liver if the tumor has not spread to other body parts. Sometimes it's best for the surgeon to remove the whole liver. If so, the child will need a liver transplant.

Chemotherapy

Chemotherapy often shrinks the rhabdoid tumor, but usually can't wipe it out completely.

Radiation

Radiation is used along with surgery or chemotherapy, but is rarely used by itself as a first treatment.

Radioembolization with Y90

Radioembolization Y90 uses radiation (Y90) and embolization (blocking blood vessels) to treat tumors and block the blood supply to the cancer cells.

Sometimes, the family is asked to participate in a clinical trial to compare two treatments to see which works best.

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. The care team is there to support your child and the whole family. They also can recommend websites, groups, and organizations that help families dealing with a cancer diagnosis.