Rhabdoid tumours of the brain

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Rhabdoid tumours may also be called teratoid tumours or atypical teratoid rhabdoid tumours (ATRTs). They are rare in children, but they can sometimes start in the brain or spinal cord. They usually develop when a child is younger than 3 years of age.

Rhabdoid tumours of the brain usually start in the cerebellum or brain stem, but they can happen anywhere in the brain or spinal cord. They are usually high grade, tend to grow fast and usually spread through the central nervous system (CNS).

Treatments for rhabdoid tumours

The healthcare team will suggest treatments based on your child's needs and work with you to develop a treatment plan. Treatment for rhabdoid tumours depends on:

  • the size and location of the tumour
  • how far the tumour has grown or spread
  • if the tumour can be surgically removed (is resectable)
  • your child's age, neurological condition and general health

Newly diagnosed rhabdoid tumours are treated with surgery, chemotherapy and radiation therapy.

Recurrent rhabdoid tumours have come back after treatment. They may be treated with chemotherapy or radiation therapy. Treatment may also be offered through a clinical trial.

The following are treatment options for rhabdoid tumours.

Surgery

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 is usually given after surgery to kill any remaining cancer cells and lower the risk that the cancer will come back. Sometimes radiation therapy is also given after surgery.

Find out more about surgery for childhood brain and spinal cord tumours.

Chemotherapy

Chemotherapy uses drugs to destroy cancer cells. High-dose chemotherapy is usually given after surgery for childhood rhabdoid tumours of the brain.

Chemotherapy drugs that may be used in various combinations include:

  • cyclophosphamide
  • cisplatin
  • etoposide (Vepesid)
  • vincristine
  • carboplatin
  • ifosfamide (Ifex)
  • doxorubicin
  • methotrexate
  • thiotepa (Tepadina)
  • cytarabine (Cytosar)

Find out more about chemotherapy for childhood brain and spinal cord tumours.

Radiation therapy

Radiation therapy uses high-energy rays or particles to destroy cancer cells. It is not used in children younger than 3 years of age because it can damage developing brain cells. In older children, it may be used along with chemotherapy to treat rhabdoid tumours of the brain.

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.

Find out more about radiation therapy for childhood brain and spinal cord tumours.

Stem cell transplant

A stem cell transplant replaces stem cells. High-dose chemotherapy followed by a stem cell transplant, or rescue, is often used to treat rhabdoid tumours.

Find out more about stem cell transplant for childhood brain and spinal cord tumours.

Clinical trials

Children with cancer may be treated in a clinical trial. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.

Expert review and references

  • Donna Johnston, MD
  • Nirav Thacker, MD
  • PDQ® Adult Treatment Editorial Board. Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment (PDQ®)–Patient Version. Bethesda, MD: National Cancer Institute; 2022: https://www.cancer.gov/.
  • PDQ® Adult Treatment Editorial Board. Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment (PDQ®)–Health Professional Version . Bethesda, MD: National Cancer Institute; 2022: https://www.cancer.gov/.
  • Geller JI. Malignant Rhabdoid Tumoura Medication . eMedicine/Medscape; 2021: https://emedicine.medscape.com/.
  • Geller JI. Malignant Rhabdoid Tumour Treatment and Management . eMedicine/Medscape; 2021: https://emedicine.medscape.com/.
  • Geller JI. Malignant Rhabdoid Tumour . eMedicine/Medscape; 2021: https://emedicine.medscape.com/.

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