What is Rhabdoid Tumor?

Malignant rhabdoid tumor (MRT) is a very aggressive form of tumor originally described as a variant of Wilms' tumor, which is primarily a kidney tumor that occurs mainly in children. MRT was first described as a variant of Wilms' tumor of the kidney in 1978. Malignant rhabdoid tumors (MRT) are a rare and highly malignant childhood neoplasm. Later rhabdoid tumors outside the kidney were reported in many tissues including the liver, soft tissue, and the central nervous system. Several cases of primary intracranial MRT have been reported since its recognition as a separate entity in 1978. The term "rhabdoid" was used due to its similarity with rhabdomyosarcoma under the light microscope. The exact pathogenesis of MRT is unknown.

Rhabdoid tumor is a rare tumor that was first found to be in the kidney. Over the last several years, research has shown that it can also occur in the central nervous system (brain and spinal cord) as well as other locations outside the kidneys such as the liver, muscle, heart, lung, soft tissues, and skin.
In the United States, rhabdoid tumor of the kidney occurs only in about 5-10 children each year, so it is extremely rare. Most children are younger than 3 years old when they are diagnosed and only rarely are rhabdoid tumors diagnosed in children older than 5.

What are symptoms of Rhabdoid Tumor?

  • Having a fever is common with rhabdoid tumors of all locations
  • Rhabdoid tumor of the brain:
  • The child's head might appear larger and the child can feel as if something is pushing on his or her head
  • Neurological changes (brain sensory and output is affected)
  • Seizure
  • High blood pressure
  • Raised intracranial pressure which means the tumor is blocking the normal flow of spinal fluid, resulting in headaches, morning vomiting, lethargy and disturbances in walking


How is Rhabdoid Tumor diagnosed?

  • CT scan (computed tomography scan) is like an x-ray that produces a set of detailed cross-sectional images of the inside of the body. The machine surrounds the body so that it can rotate around the body taking several pictures. These pictures are then put together to make a three-dimensional image of a slice of the body.

    A CT scan of the tumor site will show how far spread the tumor has grown. Once the doctor has the CT scan images, they can use these images to guide them in removing the tumor.

  • MRI (magnetic resonance imaging) scans use radio waves and magnets to create detailed cross-sectional images of the inside of the body. The body tissue absorbs the energy given off by the radio waves, and then releases the energy, which is measured by a computer to create detailed images. MRI scans may take a little longer and are usually noisier than CT scans. The machine looks like a tube, in which your child lies in, and makes a thumping noise while scanning.

    MRI scans of the abdomen may be used in addition or in place of the CT scan. MRI scans create images that tend to be more detailed than the images given by CT scans.

    MRI scans are often done to look for tumors in the arms and legs.


How is Rhabdoid Tumor treated?
The treatment for patients with rhabdoid tumor consists of the combination of surgery, chemotherapy and radiation. The current recommendations are to remove the tumor first, then continue with chemotherapy and radiation.

Tumors that cannot be completely removed or that have spread to multiple places should be biopsied first. Following a biopsy of the tumor, chemotherapy is given to shrink the tumor. Re-evaluation for surgery is done at a later time.

Radiation-high-energy rays from a specialized machine to damage or kill cancer cells and shrink tumors.


Chemotherapy-a drug treatment that works by interfering with the cancer cell’s ability to grow or reproduce. Chemotherapy is introduced to the bloodstream and travels throughout the body to kill cancer cells.

Chemotherapy can be given:

  • intravenously IV
  • intrathecally (directly into the spinal column with a needle)
  • as a pill to swallow
  • as an injection into the muscle or fat tissue


What is the Prognosis of a Rhabdoid Tumor Patient?
Regardless of location, all rhabdoid tumors are highly aggressive, have a poor prognosis, and tend to occur in children less than two years of age.