Primitive neuroectodermal tumour of the brain (PNET)

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What is Primitive neuroectodermal tumour of the brain?

Primitive neuroectodermal tumour of the brain (PNET)

Primitive neuroectodermal tumours (PNET) form a rare group of tumors defined by their appearance - they consist of small round cells in the nervous system. They are thought to develop from primitive or undifferentiated (having the potential become many different types of cell) nerve cells in the brain.

Who gets Primitive neuroectodermal tumour of the brain?

Incidence is uncertain because PNETs are rare. They are usually described as a 'tumour of childhood'. In one study the average age of diagnosis was between 3 and 8 years. Although cancer is rare in children, brain tumors are the most common type of childhood cancer other than leukemia and lymphoma.

Predisposing Factors

Researchers have not been able to find an identifiable cause or risk factors for PNET. Most cases of PNET appear to happen spontaneously as the result of a mutation in the DNA.There doesn't appear to be a genetic predisposition, meaning the disease does not seem to run in families. Certain conditions have an increased association with PNETs. These include: Li-Fraumeni syndrome, Turcot syndrome and Gorlin syndrome.

Progression

Tumours invade and destroy normal tissue, causing damage to sensory and cognitive functions of the brain. Increased intracranial pressure may result from the enlarging tumour mass, cerebral oedema (accumulation of fluid in the brain caused by swelling) and the obstruction of cerebrospinal fluid drainage (fluid that circulates throughout the brain and spinal cord).

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