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The Sydney Children's Hospital Foundation is endorsed as a deductible gift recipient by the Australian Taxation Office. If you wish to have your donation receipted, please send an email detailing the amount donated, date, name and address along with the reference BWILLSD.

About Brain Tumours in Children

Every year about 2,000 Australian children under the age of sixteen are diagnosed with a brain tumour. The most common presenting symptoms are a history of flu-like symptoms, frequent headaches that are worse in the morning and often accompanied by nausea and/or vomiting, a wobbly walk, a decline in school performance and changes in vision. Symptoms differ according to the location of the tumour. 


What is a tumour and why are brain tumours different?

A tumour is any abnormal mass in or on the body that is caused by an accumulation of cells dividing and increasing in number, in an uncontrolled way. Brain tumours can be benign (non-cancerous) or malignant (cancerous). They are among the most common types of childhood cancers and yet remain among the most difficult to cure. 

Treatment of paediatric brain tumours is more complex than that for other childhood cancers. Surgery to remove the tumour is not always possible because it may be inaccessible or because surgery would cause too much damage to the surrounding brain tissue. Even a benign tumour in the brain can be life-threatening and the prognosis for children diagnosed with brain tumours depends not only on the type, grade and size of the tumour, but on its location in the brain. 


Common childhood brain tumours 

Over 60% of childhood brain tumours are located in the posterior fossa, the lower region of the brain. This includes the cerebellum and the brain stem. Tumours in this area include medulloblastoma, cerebellar astrocytoma, brain stem Glioma and ependymoma. 

The remaining 30 to 40% of brain tumours occur within one of the two cerebral hemispheres or in the spinal column. Tumours of the hemispheres include astrocytoma, craniopharyngioma, choroid plexus papilloma, ependymomas and germ cell tumours. The most common tumours of the spinal cord are astrocytomas and ependymomas.

Brain Stem Gliomas:

Brain stem gliomas are a group of tumours located in the area of the brain called the brain stem. These account for 10% of all brain tumours in children and commonly affect children between the ages of 5 and 10. They can be low or high-grade, however a larger number (70%) are high-grade. 

The brain stem is the area of the lower back portion of the neck which connects the spinal cord with the brain. It is the area of the brain and spinal cord that is responsible for many of the body’s vital functions, including vision, swallowing, gagging, balance and strength. Because of the location of these tumours, they are more difficult to treat than tumours in other parts of the brain. 

Low-grade brain stem gliomas are focal, or confined to one area while high-grade brain stem gliomas tend to be diffuse, or infiltrate and spread throughout the brain stem. 

Children with low-grade brain stem gliomas commonly present with a long history (greater than 6 months) of symptoms including:

  • Headaches

  • Vomiting

  • Visual disturbances

  • Unsteady, clumsy walk

 

Low-grade brain stem tumours may be partially or incompletely removed, despite being located in a vital area of the brain. This is because they are generally contained to one area of the brain stem. Additional treatment such as chemotherapy and/or radiotherapy following surgery is often not required. Chemotherapy may be necessary in children whose tumour progresses following surgery or whose tumours are not surgically accessible. 

High-grade brain stem tumours because of their location within the brain stem which is diffuse and infiltrating cannot be surgically resected. Surgery in this situation could lead to damage to the nerves within the brain stem that are vital for breathing, swallowing, consciousness, arm, leg and eye movement. A biopsy is rarely performed for similar reasons. Therefore diagnosis is often made on clinical presentation and an MRI. 

Radiation treatment to the area of the tumour is the current standard treatment for this tumour type. A large percentage of children will clinically improve with radiation, but their condition will decline rapidly within 6 to 9 months after completing therapy. There is currently no clear role for chemotherapy in the treatment of these tumours. 

 

Medulloblastoma (PNET)

Medulloblastoma/PNET is the most common brain tumour of childhood, representing about 20% of all paediatric brain tumours. They are most common in children between 4 and 10 and are slightly more common in boys than girls. 

These tumours typically arise in the middle of the cerebellum, interfering with the flow of cerebrospinal fluid and causing hydrocephalus. Medulloblastoma can spread to other parts of the brain through the cerebrospinal fluid. 

Common symptoms include:

  • Vomiting (most common) with/out nausea, most often in the morning after walking

  • Headaches

  • Clumsiness

  • Difficulty with tasks like handwriting

  • Gradual decline in school performance (impaired attention)

 

If there is spread to the spinal cord symptoms may include:

  • Back pain

  • Difficulty walking

  • Problems with bowel and bladder control 

Treatment for Medulloblastoma is usually surgical removal, followed by radiation of the entire head and spinal cord and/or chemotherapy. 


Ependymoma:

Ependymomas arise from the cells that line the brain’s fluid spaces, known as the ventricles. These represent about 10% of all brain tumours in children. About 60% of children diagnosed with ependymomas are under 5. Like other brain tumours, the cause remains unknown. 

Ependymomas commonly occur in the posterior fossa (lower back of the head) part of the brain but can also occur in the top part of the brain, known as the cerebral hemispheres. 

Symptoms of tumours in the posterior fossa include:

  • Vomiting and nausea due to increased intracranial pressure

  • Headaches

  • Unsteady walking 

Symptoms of tumours in the cerebral hemispheres include:

  • Visual disturbance

  • Headaches

  • Possible seizures 

Ependymomas, like other brain tumours are diagnosed by MRI, however a biopsy is necessary to confirm this. Other tests such as a lumbar puncture can help in staging the disease. 

Tumours that arise in the posterior fossa are more often difficult for the neurosurgeon to completely remove compared to those that occur in the top areas of the brain. This is because the have the tendency to spread into the vital part of the brain known as the brain stem. This is a delicate part of the brain that must be preserved. 

Following surgery to the posterior fossa, children over the age of 4 will receive localise radiotherapy to the original location of the tumour and a small margin surrounding it. These children do not require further treatment other than frequent MRI scans to monitor for recurrence. Children who have partial or incomplete removal of their tumour will receive a course of chemotherapy following radiation treatment. 

When ependymomas arise in the top part of the brain, the neurosurgeon is generally able to remove the entire tumour without causing damage to the surrounding tissues. In this group, no additional therapy is performed and the child undergoes regular MRI scan to monitor for recurrence. 


Astrocytoma Glioma:

Astrocytomas are tumours that arise from brain cells called astrocytes. Gliomas originate from glial cells, also called astrocytes. The terms are often used interchangeably. Astrocytomas account for the majority of childhood brain tumours. In children more than 80% of astrocytomas are low-grade and 20% high grade. 

Low-grade astrocytomas are usually well localised and grow slowly over a period of time. High-grade astrocytomas tend to grow rapidly and infiltrate healthy brain tissue surrounding the tumour. These tumours require more intensive treatment. 

The signs and symptoms children present with or continue to experience are dependent upon the location of the tumour and the age of the child. The most common area for these tumours to occur is within the cerebellum, above the back of the neck. Symptoms include:

  • Headaches

  • Vomiting and nausea occurring most commonly in the morning

  • Changes in coordination

  • Unsteady, clumsy walk 

Less frequently these tumours occur in the cerebral hemispheres (left and right) at the top of the brain. Children may experience:

  • Seizures

  • Weakness in arms and legs 

Children who present with a tumour in the area of the optic nerve and hypothalamus often present with:

  • Visual disturbance (decrease in vision and double vision)

  • Hormone problems

  • Headaches

  • Arm and leg weakness 

Pilocytic Astrocytoma (low-grade astrocytoma) is a slow growing tumour with fairly well-defined borders. If the tumour is in an accessible location, the neurosurgeon will attempt a complete surgical removal. If this is achieved, no further treatment is required. Long-term survival for a totally resected pilocytic is 90% at 25 years from diagnosis. If only partial resection is achieved, further treatment may be necessary. This is aimed at partially shrinking or halting the tumour growth and includes a course of chemotherapy and possibly radiation treatment in the long-term. 

Anaplastic Astrocytoma (high-grade astrocytoma) tends to be more locally aggressive compared to pilocytic astrocytoma. Some are more responsive to treatment than others. The goal of surgery is for a complete resection without harm to the surrounding tissue. Chemotherapy and radiotherapy are needed to treat this type of tumour. 

Glioblastoma Multiforme (GBM) is a high-grade tumour representing approximately 9% of brain tumours in children.  They contain a mixture of cell types, hence the word multiforme. GBM’s are treated with aggressive surgical removal followed by intensive chemotherapy and radiation treatment to the area of the brain where the tumour originated.