Neuroblastoma

Neuroblastoma

Signs and symptoms of Neuroblastoma

The symptoms vary depending on where your child’s tumor is.  The first symptoms are vague, such as tiredness, loss of appetite and pain in the bones. More specific symptoms will depend on where the Neuroblastoma starts:

 

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If the tumor is in the abdomen, your child’s tummy may be swollen and they may complain of constipation or have difficulty passing urine.

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If the tumor affects the chest area, your child may be breathless and have difficulty swallowing.

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If the tumor occurs in the neck, it's often visible as a lump and occasionally affects breathing and swallowing.

 

 

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Occasionally, there are deposits of Neuroblastoma in the skin that appear as small, blue-coloured lumps.

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If the tumour is pressing on the spinal cord, children may have weakness in the legs and walk unsteadily. If your child is not yet walking, you may notice reduced leg movements. They may also have constipation or difficulty passing urine.

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Very rarely, children may have jerky eye and muscle movements, and general unsteadiness associated with the Neuroblastoma.

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Your child may also have high blood pressure.

How Neuroblastoma is diagnosed

A variety of tests and investigations may be needed to diagnose Neuroblastoma. These include a biopsy, blood and bone marrow tests, x-rays, CT or MRI scans, and special nuclear medicine scans called MIBG. The tests are done to find out if your child definitely has Neuroblastoma and what the exact position of the original tumor site is within the body. The tests will also find out whether the Neuroblastoma has spread.

The stage of a cancer is a term used to describe its size and whether it has spread beyond its original site. Knowing the particular type and stage of the cancer helps the doctors to decide on the best treatment for your child. 

A commonly used staging system for Neuroblastoma is described below.

Stage 1

The cancer is contained within one area of the body (localised) and there's no evidence of it having spread. It can be completely removed by surgery, or there may be very small (microscopic) amounts of tumour left after surgery.

 

Stage 2A

The cancer is localised and has not begun to spread, but it cannot be completely removed by surgery.

Stage 2B

The cancer is localised and has begun to spread into nearby lymph nodes.

 

Stage 3

The cancer has spread into surrounding organs and structures, but has not spread to distant areas of the body.

 

Stage 4

The cancer has spread to distant lymph nodes, bone, bone marrow, the liver, the skin or other organs.

Stage 4S (also called special Neuroblastoma)

This is found in children under one year old. The cancer is localised (as in stage 1, 2A or 2B) but has begun to spread to the liver, skin or bone marrow.

 

Stage L1

The tumour is localised and has not spread into important areas (vital structures) nearby. It can be removed by surgery.

Stage L2

The tumour is localised but has 'image-defined risk factors' and can't be safely removed by surgery.

 

 

 

 Stage M

The tumour has spread to other parts of the body.

Stage MS

The tumour has spread to the skin, liver and/or the bone marrow in children younger than 18 months old.

If the cancer has spread to distant parts of the body, this is known as secondary or metastatic cancer.

If the cancer comes back after initial treatment, this is known as recurrent or relapsed cancer.

 

Treatment for Neuroblastoma

The treatment of Neuroblastoma depends on the age of the child, the size and position of the tumor, the tumor biology (including the MYCN status) and whether the Neuroblastoma has spread. 

 

Surgery

For tumours that have not spread (localised tumours), the treatment is usually surgery. If the tumor is at an early stage and there's no evidence that it has spread to the lymph nodes or any other parts of the body, an operation to remove the tumor, or as much of it as possible, will be done.

A cure is usually possible for children with localized tumors. However, if the tumor is classed as high-risk due to the tumor biology results, further treatment with chemotherapy and possibly radiotherapy will be needed. If the tumor is, at first, too large or in too difficult a position to remove safely, chemotherapy will be given to shrink it before surgery.

Chemotherapy

If the tumor has already spread by the time of diagnosis, or is indicated as being high-risk by the tumor biology result, intensive chemotherapy is needed. Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It's usually given as a drip or injection into a vein. Your child's specialist will discuss with you the type and amount of chemotherapy needed.

 

High-dose chemotherapy with stem cell support

If the Neuroblastoma has spread to several parts of the body, or is high-risk with MYCN amplification, high-dose chemotherapy with stem cell support is used after the initial courses of chemotherapy.

 

 

 

High doses of chemotherapy wipe out any remaining Neuroblastoma cells, but they also wipe out the body's bone marrow, where blood cells are made. To prevent the problems this causes, stem cells (blood cells at their earliest stages of development) are collected from your child through a drip before the chemotherapy is given. These stem cells are then frozen and stored.

After the chemotherapy, the stem cells are given back to your child through a drip. They make their way into the bone marrow, where they grow and develop into mature blood cells over a period of 14-21 days.

 

Monoclonal antibody treatment

Monocolonal antibodies can destroy some types of cancer cells while causing little harm to normal cells. A new monoclonal antibody treatment called anti-GD2 is currently being tested in people with high-risk Neuroblastoma. Children in the UK with high-risk Neuroblastoma are being given anti-GD2 as part of a clinical trial. There is good evidence from a clinical trial carried out in America in 2009 that this may be a promising therapy when given alongside other standard treatment for Neuroblastoma.

Radiotherapy

External radiotherapy may be given if the Neuroblastoma is high-risk, or has spread to several parts of the body. This uses high-energy rays to destroy the cancer cells, while doing as little harm as possible to normal cells. External radiotherapy is given from a machine outside the body.

Internal radiotherapy may sometimes be given using radioactive MIBG. Radioactive MIBG is similar to the MIBG used in an investigation to diagnose a Neuroblastoma (see above), but uses higher doses of radioactivity to kill the cancer cells.

Follow-up

Follow-up after treatment usually involves regular visits to the hospital outpatients department, with scans and urine tests as necessary. For children who have had chemotherapy and/or radiotherapy, more specialised tests may be carried out. For example, hearing tests, kidney and heart function tests, and checking hormone levels. These will be repeated until your child has grown up.

If you have specific concerns about your child’s condition and treatment, it's best to discuss them with your child’s doctor, who knows their situation in detail