ATRT, Atypical teratoid / rhabdoid tumours, information for parents

Atypical teratoid / rhabdoid tumours (AT/RT): Information for parents and carers

About atypical teratoid/ rhabdoid tumours

Rhabdoid tumours that occur in the brain and spine (central nervous system) are known as atypical teratoid / rhabdoid tumours or AT/RT for short.

AT/RTs are a very rare type of brain and spinal tumour, forming only about 1 to 2% of brain tumour diagnoses in children. This means for every 100 brain tumours that are diagnosed in children, only one or two of them would be AT/RTs.

Atypical Teratoid /Rhabdoid Tumours are a type of cancer, and typically grow very fast meaning children with these tumours may develop symptoms quickly over weeks or even days.

AT/RTs usually are found in infants and young children under 3 years of age, but can occur in older children and occasionally adults as well.

Symptoms that children or infants may present with

Headaches on waking in the morning (often relieved by vomiting). In babies, this may present as morning fussiness
Nausea and vomiting, especially in the morning
Your child being unusually sleepy or unable to do skills that they had mastered a few weeks ago
An increase in head size in babies
Blurred or double vision
In older children, they may have had problems walking or losing balance. They may have also appeared to be less coordinated.

Your child’s symptoms will vary dependent on the location of the tumour. Around a half of these tumours develop in the cerebellum or brain stem (areas towards the lower rear part of the brain).

The cerebellum works to control movement, posture and balance.

The brainstem is in charge of vital functions such as breathing, heart rate and other nerves and muscles that are used for vision, hearing, walking and talking.

What investigations may my child need?

It is very important that your child has all the appropriate investigations because accurate diagnosis helps decide on the best possible treatment approach for your child.

Investigations your child may have already had include:

CT (computerised tomography) or MRI (magnetic resonance imaging) or both, usually of the brain and spine

Lumbar puncture - This removes a small sample of cerebrospinal fluid (CSF). This is then analysed to assess whether any tumour cells are present in the central nervous system (spine). This is usually done under sedation or a general anaesthetic.

An ultrasound of the abdomen and surrounding areas to check that other organs are healthy and there is no evidence of other tumours

Your child will also need other tests including:
- blood tests
- ECG’s (heart tracing)
- Cardiac echo (an ultrasound of the heart looking at how it looks and functions)

A biopsy of the tumour or the tumour may have been removed during surgery and the diagnosis of a rhabdoid tumour made following your child’s tumour removal.

Some children may need a bone scan as well.

Often, when an AT/RT is diagnosed, the doctor may have difficulty removing the whole tumour because often it may have spread by the time of diagnosis. This is the case for around 1 in 3 children with AT/RT at diagnosis.

After tissues are removed, they undergo immunohistochemistry (using antibodies to look for specific antigens using dyes) helping tell the difference between AT/RT and other brain tumours.

For information on central venous access, please see: Ports and lines

Staging of atypical teratoid / rhabdoid tumours

Staging is an assessment that doctors do for a number of forms of cancer to assess their location and whether they have spread to any nearby or distant locations in the body.

There is currently no standard staging system for this tumour type. Instead, they are categorised either as:

Localized - occurring in only one location in the brain or
Disseminated – having spread to multiple locations in the brain, spinal cord or body (around 1 in 3 children at diagnosis).

They are also categorised as to whether they are a new diagnosis or if there is recurrent disease (reoccurrence of an old tumour).

It is also noted whether all or part of the tumour is able to be removed during surgery.

What treatment may my child be offered?

Treatment will normally take place in a specialist care centre that is experienced in treating children with cancer.

There are four main treatments that are usually used in combination: surgery, chemotherapy, radiation therapy (dependent on the age of the child) and high dose chemotherapy with stem cell transplant. Usually surgery is the first step of treatment.

Often, treatment for AT/RTs is given as part of a clinical trial to give the child access to the newest possible treatment options and to tailor the best possible treatment for your child.

Surgery is often the first step in treating AT/RTs. Depending on the location and size, the surgeon may be able to remove part of the tumour. Occasionally, if surgery after biopsy is not feasible, a preoperative course of chemotherapy may be advised.

Chemotherapy is generally given following surgery. Atypical teratoid / rhabdoid tumours tumours typically are aggressive and can become resistant to chemotherapy quickly. To help reduce this chance, a combination of chemotherapy drugs are given. For more information on chemotherapy, see chemotherapy for rhabdoid tumours.

Radiotherapy: Children that develop atypical teratoid/ rhabdoid tumours may have radiotherapy depending on their age at diagnosis.

Generally, cranial radiotherapy is avoided in children under 6 months of age and used with caution in children under 3. However, it does improve survival rates of children with AT/RT so this may be offered even to very young children.

Supportive care

Treatment for childhood AT/RT can result in a number of side effects. Please be assured that the healthcare team will work carefully keep your child as comfortable and as symptom free as they can during treatment. This is called supportive care, and includes treatment for infections, pain relief and treatments to reduce side effects such as sickness.

In many areas, as a matter of routine, all children with cancer are referred to the local children’s hospice team to help with supportive care (symptom management) whilst on active treatment. This, as mentioned, is a routine but very beneficial part of a child’s treatment as it can help make them more comfortable whilst on treatment.

After treatment - follow up

Children who have been in hospital for long periods can find it difficult when they go home as they try to settle back in to everyday life, and it can take time to transition.

You and your child may also feel anxious that suddenly the number of appointments you are attending have dropped meaning you get less reassurance from staff. Don’t worry, this is all extremely common.

Understandably parents also fear the return of their child’s symptoms, meaning their cancer may have relapsed. If you have any worries please do contact your team for help and support. If your child does relapse, sometimes there are different treatment options available to try including trials, but unfortunately, this is not the case for all children.

Prognosis

How your child will do is something that doctors cannot predict. They are however aware of which children generally do better or worse than others. Certain tumour factors therefore have an impact on the chance of recovery including:

The age at which they were diagnosed
Amount of tumour remaining following surgery

Whether the tumour has spread elsewhere, for example other parts of the brain or spinal cord.

AT / RT - long term

Every child is different and will respond to treatment in a different way.

Statistics tell us the average outcome if you take a certain number of children with the same condition. They tell us the number of children that will do well and the number that will not do so well on average.

However, it is impossible to tell how each individual child will do.

This is why it is important to remember your child is an individual and may not follow the course of treatment or outcome that has been predicted.

What we know:

Overall, malignant rhabdoid tumours unfortunately do not have good survival rates.

This is especially the case if the child has signs of tumour spread at diagnosis.

Children that develop rhabdoid tumours at less than 6 to 12 months of age are less likely to do quite so well as older children.

Most rhabdoid tumours are diagnosed in infants and very young children.

We do not know what the exact outcome of each child will be.

Despite children with rhabdoid tumours generally having unfavourable survival rates, it is impossible to tell which child will do well and which child will not do so well.

Statistics do not show what will happen to your child, because each child may react differently to treatment.

Your child's consultant and medical team will help give you advice and information to make the decisions needed. If you have any concerns or questions please speak to a member of your child's team.

Join our rhabdoid families support group here

Download printable pdf - AT/ RT new diagnosis

Go back to: Rhabdoid tumours, a new diagnosis (links to other resources)

Go to:

Treatment of rhabdoid tumours: what does it involve?

Useful resources and support

When a cure may no longer be possible for my child

This information sheet will concentrate on AT/ RT and can be used alongside any other information you may have been given by your child’s team, including the CCLG publication, “My child has a brain or spinal tumour.” This publication can give you a lot of general information about what to expect during and following treatment.