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Several times ago I read some news about the death of a 6-years-old child because of cancer called neuroblastoma. I felt so sad because he is still a young kid, so I think I should find more about neuroblastoma and here is what I found.

Neuroblastoma is a cancer of specialised nerve cells, called neural crest cells. These cells are involved in the development of the nervous system and other tissues.

Fewer than 100 children are diagnosed with neuroblastoma in the UK each year. Most children who get this cancer are younger than five years old.

Most neuroblastomas (about two thirds) start in the abdomen. About one third of neuroblastomas start in the adrenal glands and another third begin in the sympathetic nervous system ganglia of the abdomen. The rest start in sympathetic ganglia of the chest or neck or in the pelvis. Some can start in the spinal cord. Rarely, a neuroblastoma may have spread so extensively by the time it is found that doctors are unable to determine exactly where it started. Unfortunately, the cause of neuroblastoma is still unknown.

The first symptoms are often vague, such as loss of appetite, tiredness and pain in the bones. Other symptoms vary, depending on where the neuroblastoma starts:

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. Sometimes your child’s blood pressure is found to be high.

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

If the tumor occurs in the neck, it may be visible as a lump as well as affecting breathing and swallowing.

Less often, children may have weakness in the legs and walk unsteadily if the tumor is pressing on the spinal cord.

Very rarely, children may have jerky eye and muscle movements associated with the neuroblastoma.

A variety of tests and investigations may be needed to diagnose a neuroblastoma. Tests such as x-rays, CT or MRI scans, bone marrow tests and blood tests are carried out to find the exact position of the neuroblastoma within the body and to see whether it has spread. This is known as staging.

A specific type of urine test will also be done. Nearly all (9 out of 10) children with neuroblastoma will have substances called vanillylmandelic acid (VMA), or homovanillic acid (HVA), in their urine. Measuring the VMA and HVA in the urine can help to confirm the diagnosis. Your child will also have their VMA and HVA levels checked during treatment. The levels of these substances will fall if the treatment is working. As these chemicals are produced by the tumor cells, and can be used to measure tumor activity, they are sometimes known as tumor markers.

An mIBG (meta-iodo-benzyl guanidine) scan may also be done. mIBG is a substance that is taken up by neuroblastoma cells. It is given by injection. Attaching a small amount of radioactive iodine to the mIBG enables the tumors to be seen by a radiation scanner. mIBG may also be used as a treatment.

Small sample of cells are usually taken from the tumor (biopsy) during an operation under a general anesthetic. The cells are then examined under a microscope. Other tests, collectively referred to as tumor biology are also carried out on these cells in the laboratory.

A commonly used staging system for neuroblastoma is described below:

Stage 1 The cancer is contained within one area of the body (localized) and there is no evidence of it having spread. It can be completely removed by surgery, or there may be very small (microscopic) amounts of tumor left

Stage 2A The cancer is localized and has not begun to spread, but cannot be completely removed by surgery

Stage 2B The cancer is localized 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, liver, skin or other organs

Stage 4S The cancer is localized (as in stage 1, 2A or 2B) and has begun to spread to the liver, skin or to some extent the bone marrow. This is found in children under one year 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 cancer. The treatment of neuroblastoma depends on the age of the child, the size and position of the tumor, the tumor biology, and whether the neuroblastoma has spread.

For tumors that have not spread (localized tumors), the treatment is usually surgery.

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.

If the neuroblastoma has spread to several parts of the body, or is high risk, high-dose chemotherapy with stem cell rescue is used (after the initial courses of chemotherapy).

If the neuroblastoma has spread to several parts of the body or is high risk, external radiotherapy may be given. 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 of the body. Internal radiotherapy may sometimes be given using radioactive mIBG. Radioactive mIBG is similar to the investigation used to diagnose a neuroblastoma, but uses higher doses of radioactivity to kill the cancer cells.

Well, that is an overview about neuroblastoma. I hope it can give you more information and prevent the lateness of treatment if your child has the symptoms of neuroblastoma.

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