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Treatment for primitive neuroectodermal tumour (PNET)

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This page is about treatment for PNET brain tumours. There is information about

 

A quick guide to what's on this page

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 made fluid build up in the brain (hydrocephalus) you may need to take steroid tablets until you have an operation. The surgeon may make a hole to allow the fluid to drain into another area of the brain (called a venticulostomy) or they may put a shunt in. This reduces the swelling and fluid collection. Reducing the fluid build up reduces the pressure in the skull and helps to control the symptoms caused by the tumour. 

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 sometimes the whole of the spinal cord after surgery.

Treatment for children under 3

There are 5 main types of primitive neuroectodermal tumour. Those that occur in very young children tend to be a type called desmoplastic tumours. These tumours are very sensitive to treatment and can often be cured with chemotherapy on its own. The child has chemotherapy using a number of drugs given into a vein. They also have chemotherapy into the fluid around the spinal cord (intrathecal chemotherapy).

If the PNET comes back

For older children and adults, chemotherapy is mainly 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 of drugs.

 

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Surgery for PNETs

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. These most often occur in the cerebellum at the back of the brain. 

If the tumour has made fluid build up in 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 they may put a tube called 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.

About 1 in 4 people who have tumours in the cerebellum have particular symptoms after surgery. The symptoms are called posterior fossa syndrome and they can be very mild or more severe. The symptoms include difficulty talking (sometimes not being able to talk at all), difficulty swallowing, some difficulty with movement or walking, and emotional changes. 

These signs and symptoms of posterior fossa syndrome may develop from one day to a week after surgery. They usually improve slowly over a few weeks or months but they don't always go away completely. The cause of the symptoms is not known but researchers are trying to find out.

 

Radiotherapy for PNETs

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%) with a tumour in the back part of the brain (medulloblastoma) it has spread to the spinal cord when they are diagnosed. 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 spinal cord after surgery.

 

Treatment for children under 3

There are 5 main types of primitive neuroectodermal tumour. Those that occur in very young children tend to be a type called desmoplastic tumours. These tumours are very sensitive to treatment and can often be cured with chemotherapy on its own. The child has chemotherapy using a number of drugs given into a vein. They also have chemotherapy into the fluid around the spinal cord (intrathecal chemotherapy).

 

Chemotherapy for PNETs

Chemotherapy may be used alongside radiotherapy for older children with medulloblastoma and for adults where there is a high risk of the tumour spreading. Otherwise 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

 

More information about PNETs

If you would like more information about treatment for PNETs you may find information in our section about brain tumour treatment.

You can find information about the outlook (prognosis) for these tumours on the brain tumour statistics and outlook page

You are also welcome to contact the Cancer Research UK nurses on freephone 0808 800 4040. Lines are open from 9am to 5pm, Monday to Friday. 

You can contact one of the brain tumour organisations or look at our brain tumour reading list. If you want to find people to share experiences with online, you could use CancerChat, our online forum.

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Updated: 16 December 2013