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The treatment of malignant rhabdoid tumours (MRT) and extra renal rhabdoid tumours (ERRT) usually involves a combination of surgery, chemotherapy and often radiation, dependent on the age of the child.

 

Rhabdoid tumours are rare tumours so treatment options are usually tailored to the child’s situation as there is no standardised tumour protocol available.

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Often the treatment that is offered may be part of a trial to give your child the most up to date treatment options. Your doctor and members of the care team will discuss the options with you in depth.

 

Treatment usually needs to be aggressive to give your child the best possible prognosis.

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What treatment may my child be offered?

Surgery

Surgery (removal of the tumour) is the first step of treatment if your child's tumour is small or in a relatively accessible location. If the tumour is too large to remove, usually, chemotherapy will have been given first.

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Dependent on the size and location of the tumour, the surgeon may be able to remove part or all of the tumour.

 

For children with MRT,(renal rhabdoid) surgery almost always involves the removal of the child’s kidney. This is done though quite a large incision to ensure all or as much as possible of the tumour is removed. Samples of lymph nodes around the tumour are usually taken as well to look for any signs of tumour spread.

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For children with ERRT (extra renal rhabdoid), the surgery involved depends on the site of the tumour. If possible, your child's surgeon will try to remove the whole tumour in one go. However, this is not always possible.
 

Chemotherapy

Chemotherapy is often the first step of treatment if your child's tumour is in a difficult location or is too large to remove surgically immediately.

This is often the case, and chemotherapy can be used to help shrink the tumour.

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Chemotherapy is a group of different drugs that affects the way cancer cells grow and reproduce. As a result, they work to shrink tumours and fight the cancer cells. Although good at fighting cancer cells, chemotherapy drugs cannot tell the difference between normal healthy cells and cancer cells. As a result, healthy cells are damaged too resulting in side effects.

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Rhabdoid tumours typically are aggressive and can become resistant to chemotherapy quickly.

 

To help reduce this chance, a combination of chemotherapy drugs are given, often in alternating cycles to help fight the tumour in the most effective possible way.

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Radiotherapy

Children that develop rhabdoid tumours at over 6 months of age will usually receive radiation as part of their treatment.

 

Dependent on the location of the tumour, the child will usually receive conventional radiotherapy, but in some cases they may require proton radiotherapy. Your clinician will advise you on this.

Supportive care

Treatment for childhood rhabdoid tumours 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.

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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.

Chemotherapy for rhabdoid tumours is often intense, and may result in your child needing an (autologous) stem cell transplant. This is where some healthy cells are removed from your child before he or she receives very high dose chemotherapy and or radiation. Following this, the child is given back their healthy stem cells to enable them to build up their immune system again. Your health care team will be able to advise if this is necessary.

Supportive care enables your child to feel as comfortable as possible whilst undergoing treatment, something that is so important.

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