For Patients & Families

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Childhood cancers are rare, but there are a number of Very Rare types – VRT.

On this EXPeRT website we will discuss the following

  • Types of rare tumours in children
  • Causes of rare tumours in children
  • Treatment for rare tumours in children
  • Coping with a rare tumours

More children than ever are surviving childhood cancer. There are new and better drugs and treatments, but it remains devastating to hear that your child has cancer, and at times it can feel overwhelming. It may seem more difficult for parents and doctors when a child has a VRT, as there seems to be little information available to help with decisions about the most appropriate treatment, or for predicting the future. There are many healthcare professionals and support organisations to help you through this difficult time, and this website can give you information and support.

VRT could be considered as an “orphan” diseases, for many reasons: there has been a paucity of clinical and biological details and many pediatric oncologists/surgeons may be unaware of the treatment options available; no specific clinical or scientific organizations have been established so far to support their clinical management and related research; it is very difficult to conduct clinical trials on them, and this makes it hard to develop evidence-based treatment guidelines, so their treatment is usually individualized; dedicated financial resources have been limited.

Understanding more about the VRT your child has and the treatments that may be used can often help parents to cope. We hope you find the information here helpful. Your child’s specialist will give you more detailed information. If you have any questions it’s important to ask the specialist doctor or nurse who knows your child’s individual situation.

Types of Very Rare Tumours in children

Very rare tumours in children make up fewer than 1 in 30 of all childhood cancers and can broadly be grouped as:

  • Very rare tumours that only affect children, such as pancreatoblastoma, malignant rhabdoid tumours and pleuropulmonaryblastoma
  • Very rare tumours that usually affect adults, such as cancers of the digestive system, the thyroid and the adrenal gland such as adrenal cortical carcinoma
  • Very rare tumours in the head and neck area, such as nasopharyngeal cancer
  • Very rare hormonal/endocrine tumours, such as phaeochromocytoma
  • Very rare brain tumours, such as meningioma
  • Very rare skin tumours, such as melanomas.


Causes of Very Rare Tumours in children

The causes of most very rare childhood tumours are unknown. But if other family members have had particular types of cancer, this may sometimes suggest that there is an inherited faulty gene in the family. If this is a possibility, your child’s specialist will talk to you about it.


Treatment for Very Rare Cancers in children

VRT are treated with the same treatments used for other childhood tumours. This includes surgery, radiotherapy and chemotherapy. Your child may need a combination of these treatments.


Usually an operation is done to remove all or as much of the tumour as possible. Other treatments, such as radiotherapy or chemotherapy, may be given after or sometimes before surgery. They may also be used if an operation isn’t possible.


Radiotherapy uses high-energy rays to destroy the cancer cells, while doing as little harm as possible to normal cells. It can be used to treat any cancer cells that may be left behind after surgery, or to shrink a cancer.


Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer or tumour cells. It is usually given as injections and drips (infusions) into a vein. Chemotherapy may be used to lower the risk of the tumour coming back or to treat tumours that has spread to other parts of the body.


Coping with a Very rare tumour

It’s always a difficult time for parents when a child has a rare disease. We will work to prepare information that could help you to understand the tumor.