Children's Cancer

The brain and the spinal cord make up the central nervous system (CNS). Different types of tumours can start in the CNS. They are named after the type of cell or part of the brain/spinal cord from which they arise. Even though these cancers start in the CNS, they are all different cancers, and need to be treated differently.

This section focuses on brain and CNS tumours that start in the CNS. It doesn’t include information about cancers that spread, or metastasise, to the brain or CNS.

Glioma

Gliomas develop from glial cells. These are cells in the CNS and the peripheral nervous system that don’t produce electrical impulses. There are different types of glial cells.

Astrocytoma

Astrocytomas develop from a glial cell called an astrocyte. An astrocyte is a cell that supports the nerve cells in the brain. 

The terms ‘astrocytoma’ and ‘glioma’ are often used interchangeably. 

The most common types of astrocytomas are:

• juvenile pilocytic astrocytoma (JPA), a slow-growing tumour, usually in the back of the brain (cerebellum). This type of tumour is referred to as a low grade glioma.
• glioblastoma multiforme (GBM), a fast-growing tumour that usually starts in the upper part of the brain. This type of tumour is referred to as a high grade glioma.
• anaplastic astrocytoma, a fast-growing tumour that usually starts in the upper part of the brain. This type of tumour is referred to as a high grade glioma.

Diffuse midline glioma (DMG) includes tumours previously referred to as Diffuse intrinsic Pontine Glioma (DIPG)

Diffuse midline glioma (DMG) is characterised by K27M mutations. They are most commonly located in the brainstem and when located there used to be called diffuse intrinsic pontine gliomas (DIPGs). The brain stem, which ‘connects’ the brain and the spinal cord, is the control centre for vital body functions such as the heart beating and breathing.

Optic pathway glioma

Optic pathway gliomas can start in either the:

• optic nerve, which connects the brain with the eyes
• nerve pathways in the brain that control vision.

Ependymoma

Ependymomas start from ependymal cells. These cells line the fluid-filled areas of the brain (called the ventricles) and spinal cord. The fluid in these areas is called cerebrospinal fluid. There are many subtypes of ependymomas, depending on where they start in the brain. If they start in the upper part of the brain, they are called supratentorial ependymomas. The majority of supratentorial ependymomas are called RELA fusion–positive ependymomas. If they start in the back part of the brain, they are called posterior fossa ependymomas. This is most commonly seen in children and our understanding of posterior fossa ependymomas is evolving and will soon be split into molecularly distinct subgroups. Spinal cord ependymomas are most commonly seen in adults.

Ependymomas can spread to other parts of the CNS.

Medulloblastoma

Medulloblastomas develop from a type of nerve cell in the back of the brain (cerebellum). They tend to be fast-growing tumours and often spread to other parts of the brain or spinal cord. At the molecular (genetic) level, it is now recognised that there are four main distinct subgroups of medulloblastoma called WNT medulloblastoma, Sonic Hedgehog (SHH) medulloblastoma, Group 3 medulloblastoma and Group 4 medulloblastoma.

Rare brain and CNS tumours

There are several types of rare brain and spinal cord tumours, including:

• atypical teratoid rhabdoid tumours (ATRT)
• pineoblastoma
• CNS Embryonal tumours (this includes tumours previously referred to as primitive neuroectodermal tumours (CNS-PNET). Our understanding of this complex group of tumours is evolving and will soon be split into several molecularly distinct tumour types
• embryonal tumor with multilayered rosettes (ETMR), CNS germ cell tumours.
• Craniopharyngioma

Chance of a cure

One of your biggest concerns on learning your child has cancer may be about their chance of being cured.

Due to major advances in treatment, many children treated for cancer now survive into adulthood. Children diagnosed with cancer between 2004 and 2012 have a 5-year survival rate of 85%. In the 1980s, the 5-year survival rate for all cancers was about 73%.¹

Talk to your child’s doctor about your child’s diagnosis, treatments and long-term survival. Long-term survival is also called the outlook or prognosis. It depends on several things, including:

• age of your child at diagnosis
• extent or stage of the cancer
• how the cancer cells look under a microscope (the shape, function and structure of the cells)
• how the cancer responds to treatment
• cancer or tumour biology, which includes
  • the patterns of the cancer cells
  • how different the cancer cells are from normal cells
  • how fast the cancer cells are growing.

To learn more about survival for tumours of the central nervous system in children, visit Australian Cancer Childhood Statistics Online

References

[1] https://cancerqld.blob.core.windows.net/content/docs/Childhood-Cancer-in-Australia-1983-2015.pdf

Clinical trials

It’s possible that your child may be able to be part of a clinical trial. Clinical trials are research investigations to test new treatments, interventions or tests, as a way to prevent, detect, treat, or manage various diseases or medical conditions. The patients involved in clinical trials are volunteers.¹

With regard to cancer, researchers run clinical trials to test new ways to:

• treat cancer
• find and diagnose cancer
• manage cancer symptoms and treatment side effects.

If a trial is available, taking part in one will be entirely the family’s decision

It’s important to note that any new treatments are strictly regulated, and must be approved before they can be used in a clinical trial. Your child’s doctor will explain everything about the trial and give you detailed written information. If you wish your child to be part of the trial, you will need to give permission.

Participating in a clinical trial may or may not directly benefit your child. However, the results of clinical trials today help children with cancer in the future. If you’re interested in participating in a clinical trial, ask your child’s doctor if there are any suitable for your child.

You can find further information on our Clinical trials and research page.

References

[1] https://www.australianclinicaltrials.gov.au/what-clinical-trial

Diagnosis

When your child is diagnosed with cancer, it can feel overwhelming. This phase involves finding out if your child has cancer, and determining the type of cancer they have. Children’s cancer can be difficult to diagnose, as many symptoms are similar to those caused by less serious conditions, or injuries. This means that your child may need several tests and medical appointments before you receive confirmation that your child has cancer.

If your doctor thinks your child has a brain or other CNS tumour, your child may have the following tests:

• medical history and physical exam, including a neurological exam to check the nervous system
• blood tests
• urine tests
• medical imaging, such as:
  • X-ray
  • computed tomography (CT) scan
  • magnetic resonance imaging (MRI)
  • positron emission tomography (PET) scan
• biopsy
• lumbar puncture (spinal tap).

Our section, How is cancer diagnosed? explains these tests in more detail.

Staging

Doctors use staging to describe how much the cancer has grown. Some of the tests for a brain or other CNS tumour will also help to stage the tumour. Staging measures:

• where the tumour is
• how big it is
• if it has spread to other parts of the body.

Your doctor will use this information to choose the best way to treat the tumour. Staging also gives your doctor an idea of how well these treatments are likely to work (prognosis).

Childhood brain and other CNS tumours have no standard staging system. Instead, doctors base the tumour staging on a range of factors. These include the tumour’s size and where it is. Doctors then classify tumours into different risk groups.

Risk factors

A risk factor is anything that increases a person’s chance of getting a certain condition or disease. Researchers know about some risk factors that increase the chance of developing cancer. But, for most children with cancer, the cause is unknown.

What we do know is that if a child develops cancer, it’s not because of something they, or their parents did to cause it. No one is to blame if a child develops cancer.

Even if your child has a risk factor, it does not mean they will develop cancer. Many children with a risk factor will never develop cancer. Most children with cancer have no known risk factors. Even if a child with a risk factor develops cancer, the risk factor may not have had much to do with it.

Researchers don’t completely understand what causes brain and other CNS tumours. But there are some things that are linked to a higher chance of getting brain and other CNS tumours.

Family history

In rare cases, an increased chance of getting brain and other CNS tumours can run in families. This is more likely if other family members had childhood brain or other CNS tumours. When these tumours run in families, the tumour usually grows at a younger age than in children with no family history of the disease.

Knowledge of your family’s cancer history may change over time.

Brain tumour predisposing genetic conditions

Some types of genetic conditions can increase a child’s chance of getting brain and other CNS tumours. In these cases, researchers have found specific changes in one or several genes that can cause cancer. These conditions can run in families. However, they can also start in the child due to a mutation in their DNA.

Research has linked childhood brain and other CNS tumours to the following conditions:

• neurofibromatosis type 1 (von Recklinghausen disease)
• neurofibromatosis type 2
• tuberous sclerosis
• von Hippel–Lindau syndrome
• Li–Fraumeni syndrome
• Gorlin syndrome
• Turcot syndrome.

If your child has one of these genetic conditions, they will need specific care. Your health care team will talk to you about which ongoing tests your child will need.

Childhood cancers that are linked to genetic conditions may affect the risk for other family members. You can ask your child’s treatment team if you or your family should get genetic counselling.

For more information about genetic conditions, see the children’s cancer glossary or the Centre for Genetics Education.

Exposure to radiation

High doses of radiation to the head can increase a child’s risk of getting brain tumours later in life. This can also happen as a result of your child being treated for other cancers.

Support and more information

A diagnosis of cancer in a child is difficult for your family. It is normal for you and your family to feel overwhelmed, scared, anxious or angry.

If you or others around you are having trouble coping, make sure you speak to your child’s treatment team. They have helped and supported many other families who have been through what you’re currently going through. Sometimes just letting other people know how you’re feeling helps you feel less alone. If you feel supported, then you will feel more able to support your child.

Most children’s hospitals will allocate a social worker to each family to provide support throughout treatment. If you need support, contact your hospital-based social worker to let them know how you’re feeling and to see what support can be made available to you and your family, including music therapists, play therapists or education support staff.

The following webpages and organisations also offer support and/or extra information for children with cancer and their families:

• Living with children’s cancer has information about physical, emotional and practical issues during and after diagnosis and treatment.
• Organisations that can provide support and information. Brain Tumour Alliance Australia supports children with brain cancer as well as their families. The Brainchild Foundation also supports families.
• Redkite is a national organisation that provides emotional support, financial assistance, information and resources to families who have a child with cancer. You can reach them through their support line 1800 733 548 (1800 REDKITE), which is open (9am-7pm AEST), email support@redkite.org.au or live chat on www.redkite.org.au
• Canteen provides a service called Canteen Connect, an online community for young people aged 12-25 dealing with their own or a close family member’s cancer, and Parent Connect, an online community with resources, information and peer support on parenting through cancer. Online counselling is available seven days a week, including evenings. Visit https://canteenconnect.org or call 1800 835 932.
• The Cancer Council in your state or territory can give you:
• general information about cancer
• information on resources and support groups in your area.
• Call the Cancer Council Helpline from anywhere in Australia on 13 11 20.
• Any of the major children’s hospitals and networks in your state or territory can provide information about childhood cancer.

For more information about types of childhood brain and other CNS tumours, see:

• Brain and spinal cord tumors in children, from the American Cancer Society
• Treatment PDQs® from the National Cancer Institute (United States), including
  • Childhood astrocytomas
  • Childhood brain and spinal cord tumors
  • Childhood brain stem glioma
  • Childhood CNS atypical teratoid/rhabdoid tumour
  • Childhood CNS embryonal tumours
  • Childhood CNS germ cell tumours
  • Childhood craniopharyngioma
  • Childhood ependymoma
• Cure Brain Cancer Foundation
• Childhood brain tumours, from the Cancer Council NSW

Note that information from international organisations may not always apply to children in Australia.

Symptoms

Symptoms are not the same in every child, but can include:

• headaches
• nausea or vomiting
• problems with seeing (reduced and or double vision), hearing or talking
• problems with balance or coordination
• weakness or numbness in a part of the body, especially only on one side
• back pain
• changes in behaviour
• weight loss or weight gain for no reason
• seizures (fits)
• drowsiness or coma.

Very young children or babies may not be able to tell you their symptoms. Symptoms in young children can also include:

• irritability
• loss of appetite
• delays in development
• not thriving – where a child fails to gain enough weight
• decrease in physical or intellectual abilities, or losing abilities that they had before
• increase in head size or swelling of the soft spots (called fontanelle) of the skull.

Symptoms of brain and other CNS tumours can depend on:

• where the tumour is
• how fast it is growing
• the child’s age.

Some symptoms occur because the tumour creates pressure in the brain. Pressure in different parts of the brain can produce different symptoms.

Treatment

Your team of doctors, called a multidisciplinary team (MDT), will care for and treat your child, and will ensure that all your child’s needs are considered while they have cancer treatment.

Treatment for brain and central nervous system (CNS) tumours depends on:

• the age of your child
• the stage of the cancer
• other factors.

Your doctor will suggest treatment options based on your child’s situation. Treatment may involve one or more of the following:

• surgery
• chemotherapy
• radiation therapy
• targeted therapy
• other treatments
• careful observation.

See The treatment team for more information.

Surgery

Most children with brain or other CNS tumours have surgery to remove all or part of the tumour. Brain surgery can affect your child’s brain or nerves. However, the doctors (called neurosurgeons) who perform this surgery are extremely careful and highly experienced and can avoid damaging your child’s normal brain tissue.

Sometimes, the entire tumour can be removed before it has spread to other parts of the body. If this is the case for your child, they may not need any other treatment.

Surgeons may remove part of your child’s brain tumour if:

• the tumour has spread to surrounding areas
• it’s in a part of the brain or CNS that makes complete removal of the tumour very difficult.

Surgery can reduce the size of the tumour that needs to be treated with chemotherapy or radiation therapy. Surgery may make these treatments more successful.

Brain surgery may also be performed to:

• relieve symptoms such as headaches, nausea, seizures and blurred vision (if the tumour is putting pressure on the brain)
• drain excess fluid to relieve pressure on the brain
• to insert a device that allows chemotherapy to be delivered directly into the cerebrospinal fluid (known as a ventricular access catheter).

After surgery, your child may have tubes coming out of the surgical site to drain excess fluid out of the skull. These tubes are usually removed after a few days.