top of page

Extra renal rhabdoid tumours (ERRT): information for parents and carers

Download pdf printable version

About extra renal rhabdoid tumours

Rhabdoid tumours were originally recognised and diagnosed when occurring in the kidney. It was later on recognised that these tumours could occur elsewhere, such as the soft tissues, lungs and skin. These became known as extra renal rhabdoid tumours or ERRT.     

​

ERRT are usually found in infants and young children, but can occur in older children and occasionally adults as well.

​

According to statistics, there are currently thought to be about 3 cases of extra renal rhabdoid tumours diagnosed a year in the UK.

Symptoms at diagnosis

Extra renal rhabdoid tumours can present with a variety of symptoms dependent on the location and size of the tumour. It may simply present with a child complaining of pain, or your baby being more ‘fussy than normal.’

​

Sometimes an extra renal rhabdoid tumour in soft tissue or bone may present with a mass or swelling that is increasing in size.

 

They may also present with some of the following dependent on the location of the tumour:

  • Feeling tired

  • Paralysis or weakness of an arm, leg or another part of their body. This may include the muscles of the face

  • Unsteady walking or loss of balance

  • Abdominal swelling

What makes it an extra renal rhabdoid tumour?

Extra renal rhabdoid tumours (and malignant rhabdoid tumours) have characteristic changes in the cells called a SMARCB1 mutation of the INI1 gene. It is this change (or mutation) that is responsible for the development of all rhabdoid tumours. For more information, please see the information section on the genetics of rhabdoid tumours.

What investigations may my child need?

  • An ultrasound scan

  • A CT or MRI scan (computerise tomography) and/ or MRI (magnetic resonance imaging) scan or both – usually including the abdomen (tummy), chest and head.

  • A biopsy of the tumour or the tumour may have been removed during surgery

  • Bone scan in some children

  • Your child will also need a range of other tests including:

    • Blood tests

    • Chest Xray

    • ECG’s (heart tracing) 

    • Cardiac echo

  • For a more detailed explanation please see section on Investigations my child may need, explained.

Staging of extra renal rhabdoid tumours

Staging is an assessment that doctors do to assess the tumour, its location and whether it has spread to any nearby or distant locations in the body.

 

Extra renal rhabdoid tumours spread quickly, and as such, many children have signs of tumour spread at diagnosis. ERRT are usually classified as:

​

Stage 1: The tumour is limited to its site of origin. The tumour wall (the capsule) has not been damaged and it has been removed whole. The tumour has not been biopsied prior to it being removed.

​

Stage II: The tumour has extended beyond the tumour wall, but it has been completely removed. There is no evidence of tumour at the resection margins (the cut edge around the tumour removed). There is no spread to the lymph nodes.

​

Stage III: The main tumour has spread to local sites surrounding it, including:

  1. To local lymph nodes or other local structures

  2. Some tumour cannot be removed by surgery

  3. The tumour ruptures before or during surgery or has been biopsied before removal.

 

Stage IV: Metastases or lymph node spread is present to distant areas. Often this may be in the lungs, liver and brain.

What treatments may my child need?

​

Most children will be offered a combination of surgery, chemotherapy and often radiation therapy for more information see our section on treatment of rhabdoid tumours: what does it involve?

 

This is dependent on the staging of the tumour and the age of the child.

​

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.

Rhabdoid tumours - 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.

Note,

Despite children with rhabdoid tumours generally having poor 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.

​

​

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

 

Go to:

Investigations my child may need, explained.

Treatment of rhabdoid tumours: what does it involve?

Useful resources and support

When a cure may no longer be possible for my child

​

Join our rhabdoid families support group here

Download pdf printable version of information on ERRT

bottom of page