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Malignant Rhabdoid Tumours: information for parents and carers
About malignant rhabdoid tumours
Malignant rhabdoid tumours (MRT) are tumours that usually occur in the kidney. They are classed as a rare type of renal tumour that particularly affects infants and very young children.
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The average age of diagnosis is 15 months old, however, MRT can be found in older children occasionally as well. There are currently thought to be about 3 cases a year in the UK.
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Around 1 in 5 children with a malignant rhabdoid tumour (renal) also have a brain or spinal tumour at the same time.
What does a renal (kidney) tumour mean?
There are two kidneys, one on each side of the spine, just below the ribcage. The kidneys clean the blood, taking out waste and fluids as urine. The urine collects and then passes through the ureter, into the bladder, and out of the body. The kidneys also make hormones that help control blood pressure and signal the bone marrow to make red blood cells when needed.
What makes it a malignant rhabdoid tumour?
Often, on scans, malignant rhabdoid tumours look similar to Wilms and other renal tumours. It is not until the histopathology (how the cells in the tumour appear under the microscope and various chemical tests) comes back that a rhabdoid tumour diagnosis can be made.
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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 malignant rhabdoid tumours. For more information, please see rhabdoid tumors, the genetics.
Symptoms at diagnosis
Malignant rhabdoid tumours often present with signs and symptoms of a mass in your child’s abdomen (tummy).
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In very young children, pain can be difficult to assess, but often your child may have been more fussy than normal. They may also have presented with visible blood in their urine or high temperatures. Sometimes, they may have none of these symptoms.
On examination, the child may be anaemic (a low number of red blood cells) or have high blood pressure.
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What investigations may my child need?
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An ultrasound scan
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A CT or MRI scan (computerised tomography) and/ or MRI (magnetic resonance imaging) scan or both – usually including the abdomen (tummy), chest and head.
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A biopsy of the tumour or the tumour may have been removed during surgery
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Bone scan (in some children)
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Your child will also need a range of other tests including:
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Blood tests
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Chest X-ray
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ECG’s (heart tracing)
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Cardiac echo
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For a more detailed explanation please see section on Investigations my child may need, explained.
Staging of malignant 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.
Malignant rhabdoid tumours are aggressive and spread (metastasize) early meaning many children already have signs of tumour spread at diagnosis, often this spread may be seen in the lungs.
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Malignant rhabdoid tumours are generally staged as
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Stage 1: The tumour is limited to the kidney and has been completely removed. 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.
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Stage II: The tumour has extended beyond the kidney, such as into the renal capsule but it has been completely removed. There is no evidence of tumour at the resection margins (the cut edge around the tumour removed) or in lymph nodes.
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Stage III: The main tumour is confined to the abdomen (tummy) but has also involved either:
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Lymph nodes in the abdomen or other close structures
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Some tumour cannot be removed by surgery (even a small amount)
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The tumour ruptures either before or during surgery called spillage
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Tumour biopsy is performed before the tumour is removed
Stage IV: Metastases or lymph node spread is present outside the abdomen.
This may be in the lungs, liver and brain.
Stage IV is the most common stage for a child to be diagnosed with.
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Stage V: Tumours are present in both kidneys
What treatments may my child need?
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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.
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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.
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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.
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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
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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.
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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.
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What we know:
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Overall, malignant rhabdoid tumours unfortunately do not have good survival rates.
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This is especially the case if the child has signs of tumour spread at diagnosis.
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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.
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Most rhabdoid tumours are diagnosed in infants and very young children.
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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.
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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.
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Join our rhabdoid families support group here
Go back to: Rhabdoid tumours, a new diagnosis (links to other resources)
Go to:
Investigations my child may need, explained.