Rhabdoid tumours

Rhabdoid tumours are rare cancerous tumours that start in the brain and spinal cord. They occur mostly in young children (under the age of 3) but can also occur in older children and adults. Rhabdoid tumours occur more commonly in the cerebellum or brain stem, but they can occur anywhere in the brain or spinal cord. They are usually high grade, tend to be fast growing and usually spread through the central nervous system (CNS).

They may also be called teratoid tumours or atypical teratoid rhaboid tumour (ATRT).

Recurrent rhabdoid tumours are tumours that have come back after treatment.

Rhabdoid tumours are staged based on whether the tumour has spread to other parts of the brain or into the cerebrospinal fluid (CSF).

A localized rhabdoid tumour has not spread to other parts of the brain or into the CSF.

A metastatic rhabdoid tumour has spread to other parts of the brain or into the CSF.

A combination of treatments may be used to treat rhabdoid tumours.

Surgery is commonly used to treat rhabdoid tumours. Doctors will try to remove as much of the tumour as possible. Sometimes a second surgery can be done to remove more or the rest of the tumour. Even if all the tumour is removed, chemotherapy and sometimes radiation therapy are usually given after treatment to kill any remaining cancer cells and lower the risk that the cancer will come back.

Chemotherapy is usually given after surgery. Chemotherapy may be given as high-dose chemotherapy with or without intrathecal chemotherapy. Intrathecal chemotherapy is used to treat any remaining tumour or a tumour that has spread in children who are not treated with radiation therapy.

Chemotherapy drugs that may be used in various combinations include:

• cyclophosphamide (Cytoxan, Procytox)
• cisplatin (Platinol AQ)
• etoposide (Vepesid, VP-16)
• vincristine (Oncovin)
• carboplatin (Paraplatin, Paraplatin AQ)
• ifosfamide (Ifex)
• doxorubicin (Adriamycin)

Radiation is not used in children under 3 years old. In older children, it may be used along with chemotherapy.

There are 2 types of radiation therapy used. Focal radiation is focused directly on the tumour. Craniospinal radiation is given to the skull and spine. One or both types of radiation therapy may be used to treat rhabdoid tumours.

High-dose chemotherapy with stem cell transplant may be used to treat rhabdoid tumours. Stem cells are removed from the blood or bone marrow of the child or a donor and are frozen and stored. High-dose chemotherapy is then given and the stored stem cells are put back into the body after chemotherapy.

Rhabdoid tumours are more likely to come back after treatment in children under the age of 3.

Chemotherapy is commonly used to treat recurrent rhabdoid tumours. Radiation therapy may also be used if it was not used in previous treatment.

Many children with rhabdoid tumours are treated in a clinical trial. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.