About Childhood Brain Tumours

Every year in Ireland, an average of 45 children and teenagers, are diagnosed with a tumour of the brain or spinal cord. Brain tumours are the most common tumours seen in children, and account for 2 in 10 new cases of childhood cancer every year.

A diagnosis like this is of course, a very stressful time for the entire family.

This information is written for you, the parent or guardian; to provide you with information about:

  • The brain and spinal cord, which is called the central nervous system (CNS),
  • The types of tumours that occur in this area,
  • The different ways that these tumours are treated.
 
We wish to thank the doctors and nurses at Beaumont Hospital, Crumlin Children’s Hospital and Temple Street Hospital for their work on this information.
 

There are many different types of tumours and each patient is unique. This means that even the same type of tumour can affect different children in different ways, and the treatments may not be identical.

The doctors and nursing staff looking after your child will give you individual information about your child’s condition and are best placed to answer any specific questions or discuss any concerns you may have.

Diagnosis & Symptoms

Our bodies are made up of billions of tiny building blocks called cells. Each cell has a specific role to play, and contains our own DNA. In normal day to day life, our cells are constantly “turning over”, which means that they are doing their job, dying off, and being reproduced by new versions of themselves. This process sometimes results in cells with “mistakes” in them. This is common, and happens in all of us. Our immune system usually destroys cells with mistakes in them. However, for a variety of complex reasons, sometimes a cell with a mistake in it can escape from the immune system. This can result in the cell dividing and multiplying in an uncontrolled way and forming a tumour. If this happens in the brain it is called a brain tumour; if it occurs in the spine it is a spinal tumour. The name of each specific tumour, depends on the exact type of cell that grew out of control initially.

People use many different words for tumours, which can be confusing. A tumour is an abnormal group of cells forming a lump or mass. Often people may refer to a tumour as a lump or a mass. A tumour may be benign (not cancerous) or malignant (cancerous). The only way of knowing with certainty, if a tumour is benign or malignant, is by removing a piece of it (a biopsy), or all of it (a resection) and examining it in the laboratory. This is a complex process that involves many different steps. This is why it can take 7-10 days (and sometimes longer) to know an exact diagnosis.

Brain tumours that occur in infants and children are very different from adult brain tumours, both in terms of the type of cells involved and their responsiveness to treatment. Brain tumours in children usually originate there (primary brain tumours); as opposed to adults who often have brain tumours which have spread from other parts of the body (secondary brain tumours). Tumours in the brain or spinal cord can sometimes spread (metastasise), but this spread is almost always confined to within the Central Nervous System (brain and spinal cord). The cause of childhood brain tumours is unknown in the majority of cases. There are a small number of children who have a genetic predisposition for developing tumours e.g. neurofibromatosis, tuberous sclerosis, and Li-Fraumeni syndrome. If your oncologist believes that your child potentially has one of these conditions, they will discuss this with you; and together, you will make the decision, to consider testing for these conditions where appropriate. Children, teenagers or young adults who have been exposed to radiotherapy in the past have an increased risk of developing tumours in the previously irradiated site. Some radiation induced tumours are benign, and some may be malignant.

The first stage of diagnosis starts when your child is seen by your GP or local hospital. This is usually because your child has had a specific symptom, or some vague ones, that caused you concern and need to be investigated. Brain tumours are generally first discovered on a brain scan (CT or MRI). Once a brain tumour is suspected, your child is referred toa specialist team of doctors and nurses.

Neurosurgery is a very important component of the care of children or teenagers with brain or spinal cord tumours. The neurosurgeon will often be the first specialist from the specialist team to meet your family. He or she will discuss with you the symptoms you have noticed in your child and explain what was seen on the scan. It is likely that further tests/investigations/procedures are required, so that information can be gathered in a safe way; in order to explain an accurate diagnosis to you and your family.

The care of children, teenagers and young adults with tumours of the brain or spinal cord, involves a large team of specialists. We work together very closely in order to:

• Safely make an accurate diagnosis
• Create and deliver a tailored treatment plan
• Provide follow-up care after therapy
• Support the family throughout their journey

Members of The National Children’s Cancer Service (NCCS), currently based in Crumlin, together with The National Department of Neuro-surgery (currently based in Temple Street) work together to form the paediatric neuro-oncology multi-disciplinary team. This expert team includes neuro-surgeons, paediatric oncologists, radiation oncologists, radiologists, neuro-pathologists, and a dedicated nursing team. We have fostered close links with dedicated social workers, psychologists, physiotherapy, occupational therapy, speech and language therapy, dietetics, school, and complementary therapists, who provide a fundamental aspect of our service. Many other teams are involved, as their expertise is required.

Complete medical history: A doctor will sit down with you and your child and ask detailed questions about the symptoms that you have noticed recently. It is usual to also ask questions about the child’s previous medical history, birth history, family history, and developmental history etc.

Clinical Examination: Your child’s doctor will examine them at the bedside. This does not usually hurt, but some children dislike it, particularly if they are feeling unwell. Your child will have a detailed examination including a neurological examination .This involves testing reflexes, muscle strength, eye and mouth movement, coordination, and alertness.

A Computerised Tomography scan (CT or CAT scan): A CT scan is a detailed scan which looks at the whole brain. It can be used to look for tumours, swelling, bleeding and fluid problems. The CT scanner is shaped like a large Polo mint and has a table which sits inside the ring shape. Your child will need to lie still on this table. A special dye will usually be injected into their vein to give a clearer picture of their brain. A CT scan usually lasts about 15–20 minutes. For this reason younger children may need sedation or a short anaesthetic. A CT scan is associated with a small dose of radiation.

Magnetic Resonance Imaging (MRI): An MRI scan is a type of scan that uses a very strong magnet and radio-waves to give more information about the position of the tumour. Your child will need to lie still on a table in a special X-ray tunnel for 30 minutes or longer. You should prepare your child in advance for this scan as the machine is very noisy (hammering sound) and they may find it difficult to lie still in the tunnel. However, usually the children can listen to music or look at DVD to distract them until the scan is completed. Very young children are given a general anaesthetic for this type of scan. Older children who are prepared in advance tend to cope very well, but parents or guardians can also remain in the room with them during the scan. Because the scan uses magnets, all metal objects (jewellery, clips or pins) will have to be removed. If your child wears dental braces, this may make the MRI image less clear. You may be advised to have them temporarily removed for the scan. You will also need to tell staff if your child has had any metal devices implanted in their body in the 6 weeks before the scan, e.g. a metal pin for a broken leg, as the device may not yet be secure and staff may need to take special safety measures.

Ophthalmology (eye) and Audiology (hearing) examination:
Various eye and hearing tests may be performed as part of the assessment for certain tumours.

Lumbar puncture/spinal tap
A lumbar puncture involves taking a sample of fluid surrounding the spinal cord for examination. When a lumbar puncture is performed, a fine needle is carefully inserted into the lower back to take a sample of fluid. This is usually done under sedation or general anaesthetic to minimise discomfort. This test is to show if the tumour cells have spread/metastasised to the CSF. The CSF may also be analysed for chemicals called tumour markers, which certain types of brain tumours may leak into the CSF.

Bloods tests
Certain tumours called germ cell tumours release chemicals that can be found in the blood and/or CSF. If a germ cell tumour is suspected, a blood test can be taken to look for these chemicals. It is sometimes possible to diagnose the type of tumour in this way without the need for a tumour biopsy.

EEG
An EEG or electroencephalogram is a test that measures the electrical activity in the brain. The test may be done for your child if their tumour is causing seizures or fits (A fit is another word for a seizure). It involves placing small electrodes on their scalp, which transmit and record electrical brain activity onto a graph for the doctors to read. This test does not hurt, although children may complain about the sticky gel in their hair. The gel is used to help keep the electrodes in place.

Endocrine (hormone) Tests
The pituitary gland is the master hormone gland in the body, and responsible for the release of regulating hormones. Certain tumours and their treatments can sometimes interfere with the normal hormone changes that happen as your child grows. Depending on the type and location of your child’s tumour, and the type of treatment that they’ve had, they may need their hormonal system monitored closely during childhood and adolescence. This will be done at outpatient follow up clinics and usually involves physical examination and blood tests.

The vast majority of brain and spinal cord tumours require a biopsy or removal (resection) of the tumour, in order to make a diagnosis (give the tumour a name).

Biopsy
In some situations when it is not possible, or perhaps not immediately necessary to remove the tumour, doctors may need to remove a small piece of the tumour to find out exactly what it is. Your child will be given an anaesthetic for the procedure and it will be done in the operating theatre.

Tumour Resection (removal)
Sometimes, it’s necessary to remove the tumour. This may require more than one surgery. The neuro-surgical team will explain to you what type of surgery is required, the potential side-effects for your child, and the likely recovery time.

The tumour sample is then sent to the neuropathology team, who start the process of testing, to find out what type of tumour it is. A neuropathologist is a specialist doctor who examines tumour cells in the laboratory. He or she will make a diagnosis based on the sample. On average, a result can take 7 to 10 days. Sometimes the results may take longer to process depending on the complexity of the analysis. Specialist molecular (DNA profiling) results often take weeks to months to obtain. Once a diagnosis has been made all members of the specialist team work together to ensure that your child gets the most appropriate treatment in a timely way. In general, children need to recover from their surgery prior to starting other therapies.

Other Surgical interventions:
If your child’s tumour is blocking the cerebrospinal fluid (CSF) channels in their brain; this can cause an increase in pressure, which is called hydrocephalus. Hydrocephalus may result in headache, vomiting, sleepiness and abnormal eye movements. There are a variety of ways to treat this and the neuro-surgical team will recommend the most appropriate plan for your child. Hydrocephalus may be treated by tumour removal, insertion of specialised devices called shunts; or by creating a new channel called an endoscopic third ventriculostomy (ETV).

The first signs of a brain tumour are variable, and depend on many factors; including, the precise location of the tumour, the structures the tumour is putting pressure on, and the age of the child. It is very common for the initial signs and symptoms to be mistaken for more common childhood illnesses e.g. a tummy bug or migraine. We sometimes meet children or teenagers who have brain tumours and are completely well, but were having a scan of their head for another reason; this is called an incidental finding.

The HEADSMART initiative was developed in the UK, and aimed to increase population awareness regarding brain tumours in children. The symptom card (below) was designed to help you know and spot the signs and symptoms of brain tumours in children and teenagers:

Source: headsmart.org

Types of Brain Tumours

This section gives a general overview of the types of brain tumours commonly found in children and teenagers. It includes information on the type and behaviour of these tumours, a description of where the tumours occur, and the treatments generally used.
Gliomas develop from the glial (supporting cells) in the brain and make up more than half of all primary brain tumours in children. Gliomas may be named after the part of the brain in which they are found (e.g. optic pathway glioma) or after the type of cells that they contain (e.g. pilocytic astrocytoma). There are many different types of gliomas, but in general we categorise these tumours as low grade gliomas or high grade gliomas. Some children with gliomas may have an underlying condition which predisposes them to such tumours e.g. neurofibromatosis or tuberous sclerosis. Your doctor will inform you if that is suspected for your child.
Low grade gliomas are the most common type of brain tumour seen in children, representing 30-50% of all brain tumours. These tumours are typically grade 1 and 2, when examined under the microscope. These are are typically slow-growing tumours. These tumours typically cause symptoms by putting pressure on surrounding structures in the brain. The treatment of choice is to surgically remove as much of these tumours; as is safely possible. These tumours sometimes contain a cyst filled with fluid rather than solid tissue. If your child’s tumour has been completely removed, the chance of it returning is small. Your child will be monitored closely in the outpatient neuro-oncology clinic at regular intervals as occasionally these tumours can re-grow and need further treatment. If the tumour is causing symptoms or growing, further surgery, chemotherapy and/or radiotherapy may be considered. If a low grade glioma continues to grow despite surgery, chemotherapy, and/or radiotherapy; experimental or targeted therapies may be given. The treatment of a low grade glioma is a constant balance between maximizing the well-being of the patient and minimising the side effects associated with treatments.
High grade gliomas represent 8-12% of childhood brain tumours. These are malignant and tend to behave aggressively and grow rapidly. These tumours can be difficult to remove completely, as they tend to grow quickly and creep into and invade nearby normal brain tissue. The symptoms will depend on the area of the brain involved. They are usually treated by surgery followed by radiotherapy and/or chemotherapy. Types of high grade glioma include glioblastoma multiforme (GBM) and anaplastic astrocytoma

The brain stem is located at the base of the brain between the deep brain structures and the top of the spinal cord. This vital area of the brain contains many of the control centres necessary for sustaining life e.g. breathing, blood pressure and heart rate. As a result, tumours within this area can be challenging to treat. Tumours of the brain stem are usually gliomas and are usually one of two types of tumours

• Low grade glioma e.g. pilocytic astrocytoma
• High grade glioma called a diffuse intrinsic pontine glioma (DIPG); so called because it originates in the pons which is a part of the brainstem.

If safe to do so, initial surgical treatment is focused on relieving hydrocephalus (if present) and by reducing pressure effect (sometimes by giving a steroid called dexamethasone). A biopsy is performed (if considered safe to do so) to achieve a diagnosis. The treatment path followed depends on if this is a low grade glioma or a high grade glioma (e.g. DIPG). Low grade gliomas are typically treated as outlined in the glioma section (above). DIPG tumours are usually treated with radiotherapy, in an attempt to reduce tumour size, associated pressure effects and symptoms. Your child’s treating team will explain in detail the diagnosis, prognosis, treatment pathway, and will advise you of the option to participate in clinical trials, if available.

Tumours of the optic pathway are generally low grade gliomas. As the location of the tumour involves the visual pathways (nerves that carry visual information to the eyes), surgery is usually not performed to remove the tumour. If safe to do so, a biopsy is sometimes performed. A biopsy is not always required for gliomas in this area. Your child’s doctor will monitor the tumour closely with regular medical reviews, MRI scans and visual assessments (the ophthalmologist is the doctor who specialises in these visual assessments). This is called an observation strategy. If the tumour is growing or causing a major symptom for your child e.g. causing vision to deteriorate; your child’s doctor will discuss the possibility of starting treatment with chemotherapy. Occasionally radiotherapy or targeted therapies are used, depending on the particular situation.
A tectal glioma is a low-grade glioma of the tectum (the roof of the brain stem). Symptoms may vary, but most commonly include symptoms of hydrocephalus (headache, vomiting, sleepiness, changes in eye movements). Treatment is aimed at surgically relieving the hydrocephalus and does not always include a biopsy. Thereafter, the majority of children will be observed closely and will have scans and medical reviews at regular intervals. Chemotherapy or radiotherapy are seldom required for tectal gliomas that remain stable. However, if your child’s tumour is seen to be getting bigger or if more symptoms are developing, a biopsy or other treatments may be considered.

Medulloblastoma is the most common malignant (cancerous) brain tumour in childhood. In Ireland about 10 children are diagnosed with a medulloblastoma per year. These tumours often occur in or close to the cerebellum, frequently blocking the flow of CSF, and causing hydrocephalus. Initial steps in looking after children with medulloblastoma include:

• Surgical procedure to relieve hydrocephalus if present (sometimes a VP shunt is needed)
• Maximal safe surgical removal of the tumour if possible
• Post-operative MRI scan – to find out if there are tumour cells anywhere else in the brain or spine, and also to see if there is visible tumour left at the main tumour site.
• At day 10-14 after the surgery, a lumbar puncture to allow CSF testing, is carried out; in order to find out if there are any tumour cells floating in the CSF. Your child will be sedated/under anaesthetic for this to minimise discomfort.
• Bone marrow aspirates and biopsies (if there is evidence of disease spread)

The treatment of medulloblastoma depends on many factors e.g.

• Age of child
• Whether the tumour has spread or is in one place
• Whether the tumour has been totally removed or not
• The specific “molecular” subtype of medulloblastoma
• Whether the child has an underlying condition which predisposes to the development of medulloblastoma (small minority of patients)
• How well the child is after the diagnosis and surgery.

In general, treatment of medulloblastoma involves a combination of:
• Surgery
• Radiotherapy to the primary tumour site and the whole brain and spinal cord (in children over the age of 3-4 years)
• Chemotherapy

There have been a lot of scientific advances related to medulloblastoma in recent years. This has allowed us to learn more about the “molecular subtype” of medulloblastoma e.g. WNT, SHH, group 3 and group 4. In some cases this helps us to guide treatment decisions. Sometimes the treatment decisions are complex and unique to your child, however the aim is always to maximise the chance of long-term cure while minimising the chance of treatment related side-effects. Your medical team will be able to discuss this with you in more detail.

This is a rare type of embryonal tumour, which predominantly occurs in younger children i.e. two thirds of the cases are in children under the age of 3 years. This tumour can occur anywhere in the brain or spine. Very rarely, if we see that there are additional tumours outside the brain and spinal cord e.g. kidney tumours, we become concerned that a child may have a condition that predisposes to the development of “rhabdoid” tumours. If the medical team is concerned that a “rhabodid predisposition syndrome” is a possibility for your child, this will be discussed in detail with you; as not all children are affected. Initial steps in looking after children with ATRT include:

• Surgical procedure to relieve hydrocephalus if present (sometimes a VP shunt is needed)
• Biopsy or maximal safe surgical removal of the tumour
• Post-operative MRI scan – to find out if there are tumour cells anywhere else in the brain or spine, and also to see if there is visible tumour left at the main tumour site.
• At day 10-14 after the surgery, a lumbar puncture to allow CSF testing, is carried out; in order to find out if there are any tumour cells floating in the CSF. Your child will be sedated/under anaesthetic for this to minimise discomfort.
• Abdominal Ultrasound – to examine the kidneys

The treatment of ATRT depends on many factors e.g.
• Age of child
• Whether the tumour has spread or is in one place
• Whether the tumour has been totally removed or not
• Whether the child has an underlying condition which predisposes to the development of rhabdoid tumours (minority of patients)
• How well the child is after the diagnosis and surgery.

In general, treatment of ATRT involves a combination of:
• Surgery
• Radiotherapy to the primary tumour site
• Radiotherapy to the craniospinal axis (if there is metastatic disease) – in children over the age of 3-4 years
• Chemotherapy

These are malignant tumours which arise in the pineal region in the brain, which sits in the centre of the brain between the two hemispheres. The pineal gland produces a substance called melatonin which help control our sleep cycle. In the minority of children, pineoblastoma may be associated with an inherited mutation in the RB1 gene.

Initial steps in looking after children with pineoblastoma include:
• Surgical procedure to relieve hydrocephalus if present (sometimes a VP shunt is needed)
• Biopsy or maximal safe surgical removal of the tumour
• Post-operative MRI scan – to find out if there are tumour cells anywhere else in the brain or spine, and also to see if there is visible tumour left at the main tumour site.
• At day 10-14 after the surgery, a lumbar puncture to allow CSF testing, is carried out; in order to find out if there are any tumour cells floating in the CSF. Your child will be sedated/under anaesthetic for this to minimise discomfort.

In general, treatment of pineoblastoma involves a combination of:
• Surgery
• Radiotherapy (in children over the age of 3 years)
• Chemotherapy

Many other rare malignant embryonal tumours exist. Other examples include CNS-neuroblastoma, CNS-ganglioneuroblastoma, medulloepithelioma, embryonal tumour with multi-layered rosettes (ETMR) and CNS-embryonal tumour NOS (not otherwise specified). In general these tumours also require treatment with a combination of surgery, chemotherapy with/without radiotherapy (depending on the age of the child).

An ependymoma is a tumour that begins in the cells (ependymal cells) that line the ventricles of the brain and spinal cord. Ependymoma tumours vary from benign low grade tumours to malignant higher grade tumours. In recent years, new molecular discoveries provide us with more diagnostic and prognostic features of ependymoma. The low grade ependymomas (often seen in the spine) generally can be removed by surgery.

Initial steps in looking after children with higher grade malignant ependymomas involve:
• Surgical procedure to relieve hydrocephalus if present (sometimes a VP shunt is needed)
• Maximal safe surgical removal of the tumour (this sometimes requires more than one procedure)
• Post-operative MRI scan – to find out if there are tumour cells anywhere else in the brain or spine, and also to see if there is visible tumour left at the main tumour site.
• At day 10-14 after the surgery, a lumbar puncture to allow CSF testing, is carried out; in order to find out if there are any tumour cells floating in the CSF. Your child will be sedated/under anaesthetic for this to minimise discomfort.

Treatment of malignant ependymoma involves a patient specific treatment pathway:

• Surgery (Maximal safe surgical removal of the tumour (this sometimes requires more than one procedure)
• Radiotherapy: Radiotherapy only to the site of the primary tumour, in children that are over the age of 18 months. If the ependymoma has spread (rare at diagnosis), and the child is over 3 years of age, craniospinal radiation may be considered.
• Chemotherapy: Various different chemotherapy combinations and approaches are used depending on the age of the child; whether or not all the tumour was removed or not; and whether the disease has spread or not.

These tumours tend to grow in or near the midline structures of the brain e.g. the pineal gland or pituitary gland. Certain germ cell tumours release chemicals that can be found in the blood or cerebrospinal fluid (CSF). These chemicals are called tumour markers. If, after a MRI brain scan, a germ cell tumour is suspected, a blood sample and a sample of cerebrospinal fluid (CSF) can be taken to check for markers. In some cases these tumour markers allow us to diagnose the type of tumour without doing a biopsy. There are different types of germ cell tumours and are grouped into two main categories called germinomas and non-germinomatous germ cell tumours. These tumours are generally sensitive to chemotherapy and radiotherapy, but occasionally need a surgical procedure to remove parts of the tumour.

Initial steps in managing children with germ cell tumours include:
• Surgical procedure to relieve hydrocephalus if present; sometimes an endoscopic third ventriculostomy (ETV) or ventriculo-peritoneal (VP) shunt is needed.
• Blood tumour markers and CSF tumour markers
• If tumour markers are normal or not diagnostic, a biopsy may be required to diagnose the suspected germ cell tumour
• MRI Brain and Spine: to find out if there are tumour cells anywhere else in the brain or spine, and to evaluate the tumour at the primary site.
• At day 10-14 after the surgery, a lumbar puncture to allow CSF testing, is carried out; in order to find out if there are any tumour cells floating in the CSF. Your child will be sedated/under anaesthetic for this to minimise discomfort.
• Endocrinology review (hormonal assessment) may be required
• Ophthalmology review may be required

In general treatment involves a combination of:
• Chemotherapy
• Sometimes surgery (if there is tumour leftover after chemotherapy)
• Radiotherapy

Craniopharyngioma is a benign tumour found behind the pituitary gland in the midbrain. The symptoms will depend on whether the tumour is involving or pushing on the hypothalamus, pituitary gland or optic nerves. These tumours often have solid parts and cystic parts.

The type of treatment given will depend on your child’s individual case.

Treatment options:
• Surgery: the tumour is usually removed by surgery if possible. The neurosurgeons will decide which type of surgery is best for your child e.g. the tumour can sometimes be removed through your child’s nose, using a specialised surgical method, while your child is under general anaesthetic.
• Radiotherapy may be considered, in certain cases e.g. if the tumour has not been completely removed and is growing or causing symptoms.

Relieving pressure: If your child has a build-up of pressure in a craniopharyngioma cyst, they may need surgery to relieve it. Sometimes an Ommaya reservoir, or a device through which fluid can be removed from the cyst, is surgically placed under the skin to allow cyst fluid to be removed periodically.

Hormone changes: The pituitary gland is the master control centre for the body’s hormones. Craniopharyngioma and its treatment can sometimes interfere with the normal hormone changes that happen as your child grows. More than likely, your child will need their hormones watched closely during childhood and adolescence. This means that they will need blood test monitoring and careful observation of their growth and pubertal development.

Vision: Children with craniopharyngioma may have changes to their vision. This is monitored closely, and if deteriorating, may be an indication to consider further treatment.

Spinal cord tumours are relatively rare in children and teenagers, representing less than 5% of all tumours of the central nervous system. We see any of the previously described tumours in the spinal cord, but the most frequent types diagnosed include gliomas (particularly low grade gliomas) and ependymomas.

Spinal tumours may be found within the nerves of the spinal cord, or inside the coverings of the spinal cord or even outside the cord itself. They may also occur in the bone of the spine.

Symptoms depend on the position of the tumour in the spinal cord. These tumours may often cause symptoms by pressing on the nerves, including back and neck pain, numbness or weakness in one or both arms or legs. Tumours in the lower part of the spine may cause loss of bladder or bowel control.

Treatment of the spinal cord tumour depends on what type of tumour it is. Surgery will usually be performed, to allow biopsy/diagnosis or removal of the tumour or to relieve pressure on the spinal cord itself. Further treatment is dependent on tumour type.

It is outside the scope of this resource to describe every type of brain and spinal cord tumour. Other rare tumour types do occur in infants, children, teenagers and young adults e.g. choroid plexus tumours and meningiomas. Your medical team has experience in treating these tumours and will discuss them in detail with you.

Treatment

Your child will have their diagnosis and treatment plan recommended by the neuro-oncology multi-disciplinary team, which includes experts from all relevant specialties. The paediatric oncologist will discuss this treatment plan in detail with you and answer all of your questions. Each child’s treatment plan is unique to them, but will be based on “standard of care”. This means that the treatment plan which is recommended will be based on available scientific evidence and expertise, and is the “best way we know how” to treat a particular tumour type. Sometimes there is an opportunity to enrol on a clinical trial, which will be discussed with you.

Selecting treatments to treat children with brain and spinal cord tumours is always a very careful balance; that is, we aim to pick treatment which maximises our chance of cure but minimises the chances of long term effects from the tumour and the treatments. These decisions are often straightforward, but sometimes require complex discussion. Please be reassured that your medical team is committed to answering all of your questions, as we know this is a very worrying time for your family.

See above under diagnosis section.
Brain or spinal cord tumours that are not causing significant symptoms for your child, and are believed to be slow growing are often “observed” closely. This involves regular outpatient visits and MRI scans to ensure that the tumour is not showing signs of growth. There are many children who are looked after in this way, and never require intervention. If the tumour starts to grow or cause symptoms for your child, further discussion is had regarding next steps required.
Chemotherapy is a term used to describe many different types of medicines used to treat tumours (both cancerous and non-cancerous). Chemotherapy is sometimes given as a tablet or liquid in the mouth, into a vein (intravenously) or into the cerebrospinal fluid (intrathecal). Chemotherapy affects cells that are rapidly dividing, like tumour cells. The aim of giving chemotherapy is that it stops or slows down tumour growth. If a tumour has been removed completely, we often still give chemotherapy, with the aim of “mopping up” tiny tumour cells that are left over. There are many different types of chemotherapy drugs which may be used alone or in combination with others, depending on the type of tumour being treated.
Throughout the world paediatric oncologists work together to develop successful treatment plans, which are also called protocols. Children are treated on a specific protocol depending on their tumour type and age. The paediatric oncologist will explain to you in detail which protocol is suitable for your child and any possible side-effects associated with that treatment. Chemotherapy protocols are usually given over many months, with children attending the Oncology Unit at regular intervals, either as a day-patient or in-patient.
Chemotherapy is usually given by drip into a vein (intravenously) or in tablet or liquid form. For some tumours, chemotherapy is injected directly into the cerebrospinal fluid surrounding the brain and spinal cord. This is called intrathecal chemotherapy. A device called an Ommaya reservoir is sometimes used to give intrathecal chemotherapy.

Radiotherapy is a treatment that uses high-energy radiation to treat tumours. Treatment is like having an x-ray taken and is completely painless. It is usually directed very accurately at the tumour to destroy the cancer cells. Radiotherapy is given as a course of daily treatments, over a period of weeks. It is usually used in combination with surgery and/or chemotherapy. It is important for children to keep very still during treatments. The team will make a special mask to help children maintain their head in the correct position. Very young children may be treated under general anaesthetic, while older children may be prepared with play therapy and distracted during the treatment. As part of preparation, your child will have a planning CT scan, which is loaded onto a special computer programme. The radiation oncologist uses this scan, along with any previous MRIs, to find the area they will treat, along with any important organs near to the treatment area. They then produce a radiotherapy dose map, which shows the dose of radiotherapy that should be given to all the relevant areas of the brain or spine.

There are different types of radiotherapy.
• Photon radiotherapy: X-rays are most commonly used, often using a special technique known as IMRT (intensity modulated radiotherapy). This enables very accurate sculpting of the dose around the tumour while minimising the dose to the surrounding organs. This type of radiotherapy is available for children in Dublin at St. Luke’s Hospital in Rathgar.
• Proton beam therapy: This radiotherapy uses protons instead of x-rays to treat tumours. It does not improve the chance of cure, but it might reduce the risk of long-term side effects. It is not yet available Ireland, but the HSE funds proton therapy for certain types of tumour that are most likely to benefit from this option. This often takes place in Essen, Germany.

Your medical team will consider all aspects of care when selecting the best type of radiotherapy for your child – in particular, the risks of travelling and the risks of any potential delays in radiotherapy. Your medical team will discuss all of this with you in detail.

In the last decade, scientific research has led to new discoveries relating to paediatric neuro-oncology. Tumours that were thought were single entities (based on what they look like under the microscope) have turned out to be a complex family of related tumours, which often differ in the factor that is “driving” them to grow.  This has allowed doctors to learn more about the likely behaviour of a tumour type e.g. different molecular subtypes of medulloblastoma. In some instances, these discoveries have lead doctors to alter treatment plans for children, however this is usually in the context of a clinical trial, and are not front-line therapies at present. These targeted agents are sometimes available if your child’s tumour comes back (relapses) after standard treatment has been given. New information is becoming available all the time, and your medical team will be happy to discuss any potential role for targeted agents for your child’s tumour.

Izzy Leamy

Age 3, Dublin

Jake Smith

Brave & Inspirational Children

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