Read article Treatment for primitive neuroectodermal tumour (PNET)
This page is about treatment for PNET brain tumours. There is information about
Treatment for primitive neuroectodermal tumour (PNET)
The main treatment for primitive neuroectodermal tumours (PNETs) is surgery to remove the whole tumour, or as much of it as possible. If the tumour has caused fluid on the brain (hydrocephalus) you may need to take steroid tablets until you have an operation to drain the fluid. After the surgery there is a risk that the tumour may come back or spread to other areas of the brain or spinal cord. So it is important to have radiotherapy to the brain and the whole of the spine after surgery.
Treatment for children under 3
If at all possible, specialists avoid using radiotherapy to the whole brain and spine on children younger than 3. Their young age makes them more likely to develop long term side effects. So they may have chemotherapy to keep the tumour under control until radiotherapy to the whole brain is less likely to cause damage. They may have targeted radiotherapy just to the area containing the tumour.
If the PNET comes back
Chemotherapy is used to treat the tumour if it comes back after surgery and radiotherapy. PNET responds quite well to chemotherapy and doctors use a variety of different single drugs and combinations.
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The main treatment for primitive neuroectodermal tumours (PNETs) is surgery to remove the whole tumour, or as much of it as possible. The most common type of PNET is medulloblastoma. If the tumour has caused fluid on the brain (hydrocephalus) you may need to take steroid tablets until you have an operation. The surgeon may make a hole to drain fluid into another area of the brain (known as a ventriculostomy operation) or put a shunt in. This reduces the swelling and fluid collection in your brain. Reducing the fluid build up reduces the pressure in your skull and helps to control the symptoms of your brain tumour. In up to 4 out of 10 patients (40%) the shunt needs to stay in place after surgery to stop further fluid build up.
Primitive neuroectodermal tumours have a tendency to spread to other areas of the brain or spinal cord through the cerebrospinal fluid. In about 1 in 5 patients (20%) the tumour has spread to the spinal cord when they are diagnosed and in other people there is a risk that it will spread. So it is important to have radiotherapy to the brain and the whole of the spine after surgery. Newer radiotherapy techniques that are being increasingly used include conformal radiotherapy and stereotactic radiotherapy.
If at all possible, specialists avoid using radiotherapy to the whole brain and spine on children younger than 3. Their young age makes them more likely to develop long term side effects. The doctor may suggest chemotherapy to keep your child's tumour under control until radiotherapy is less likely to cause damage. The exact combination of drugs used and the timing varies.
In general, your child is likely to have high dose chemotherapy with a number of drugs, as well as radiotherapy just to the area containing the tumour. In that way, the specialist may be able to avoid giving radiotherapy to the whole brain and spinal cord. Or the radiotherapy may be delayed until your child is older.
In other situations, PNET specialists tend to keep chemotherapy to treat the tumour if it comes back after surgery and radiotherapy. PNET responds quite well to chemotherapy and doctors use a variety of different single drugs and combinations. Drugs that are used include
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