CNS PNET refers to Primitive Neuroectodermal Tumors (PNETS) of the Central Nervous System.
CNS PNETs are large tumors that grow from primitive or undeveloped brain cells.
They are found in the brain and spine and generally occur on the outside part (ectodermal). PNETS can also occur in other parts of the body however these are a different type of PNET called peripheral PNETs. This web-site focuses on CNS PNETs.
PNETs are rare and highly aggressive, grade 4 tumours.
They tend to attach to the part of the brain that controls movement, thought and sensation.
PNETs tend to strike in young children and in teenagers.
It occurs most in children. There have been cases of PNET found in older people (over the age of 30) however this is rare.
Symptoms vary according to the location and size of the tumour.
Symptoms generally include:
- Behavioural or personality changes
Unexplained weight loss or weight gain
Weakness on one side of the body
- Surgery to remove the tumour (referred to as resection)
Radiation is generally not given to children under the age of 4 or 5 and Chemotherapy is the preferred treatment in young children. The inability to give radiation in young children contributes to the lower survivor rate as there is a risk that chemotherapy alone will not be sufficient to treat the tumour.
The treatment protocol followed will depend on:
- the age of the child
- the size and location of the tumour
- the type of PNET (4 sub-types have been identified and doctors are developing tailored treatment protocols for each type)
There are a number of hospitals and clinical trials throughout the world that are focussed on developing more effective ways of treating brain cancer in children. These include:
- Proton therapy ( a special form of radiation)