Neuroblastoma

Neuroblastoma is a solid cancerous tumor that develops in the nerve tissue ofthe neck, chest, abdomen, or pelvis. The tumor usually spreads from the adrenal gland, which is located in the abdomen near the kidneys.

Neuroblastoma affects mainly children. It is the fourth most common cancer that occurs in children, affecting one in 100,000 children in the United Stateseach year. Approximately 60% of cases of neuroblastoma occur in children younger than two years old, and 70-90% occur in children under five. The diseaseis sometimes present at birth, but is usually not noticed until later. By the time the disease is diagnosed, it has often spread to the lymph nodes, liver, lungs, bones, or bone marrow.

Physicians classify neuroblastoma in stages, based on how far the disease hasspread from its original site to other tissues in the body. The best treatment for the disease depends on its stage. Neuroblastoma that is confined to the site of origin, with no evidence that it has spread to other tissues, is called localized resectable (able to be cut out) neuroblastoma. At this stage,the cancer can be surgically removed. Localized unresectable neuroblastoma isconfined to the site of origin, but the cancer cannot be completely removedsurgically. Regional neuroblastoma has extended beyond its original site, tonearby lymph nodes, organs or tissues, but has not spread to distant sites inthe body. Disseminated neuroblastoma has spread to distant lymph nodes, bone, liver, skin, bone marrow, and/or other organs. Stage IV's (or "special") neuroblastoma has spread only to liver, skin, and/or, to a very limited extent,bone marrow. Recurrent neuroblastoma is cancer that has come back or continued to spread after it has been treated. It may come back in the original siteor in another part of the body.

The most common symptoms of neuroblastoma arise because of pressure caused bythe tumor and bone pain from cancer that has spread to the bone. Cancer thathas spread to the area behind the eye may cause bulging eyes and dark circles around the eyes. Pressure on the spinal cord may cause paralysis. Fever, anemia, and high blood pressure occur occasionally. Some children may have watery diarrhea, uncoordinated or jerky muscle movements, or uncontrollable eye movements, but these symptoms are rare.

Physicians use physical examination and imaging techniques, such as computedtomography scan (CT scan) and magnetic resonance imaging (MRI), to diagnose neuroblastoma. The physician may also take a small tissue sample (biopsy) fromthe tumor or bone marrow and examine the cells under the microscope. Once neuroblastoma has been found, the physician will perform more tests to find outif the cancer has spread to other tissues in the body.

Treatments are available for children with all stages of neuroblastoma. Morethan one type of treatment may be used, depending on the stage of the disease. Surgery is used whenever possible, to remove as much of the cancer as possible, and can generally cure the disease if the cancer has not spread to otherparts of the body. Radiation therapy is often used after surgery; high-energy rays (radiation) are used to kill as many of the remaining cancer cells aspossible. Chemotherapy (using drugs to kill cancer cells) may also be used after surgery to kill remaining cells. Also, before surgery, chemotherapy is used to shrink the tumor so that it can be more easily removed during surgery.Bone marrow transplantation is used to replace bone marrow cells killed by radiation or chemotherapy. In some cases the patient's own bone marrow is removed before treatment and saved for transplantation later. Other times the bonemarrow comes from a "matched" donor, such as a brother or sister.

The chances of recovery from neuroblastoma depend on the stage of the cancer,the age of the child, the location of the tumor, and other factors. Infantshave a higher rate of cure than do children over one year of age, even when the disease has spread. In general, the prospects for a young child with neuroblastoma are good: about 85% of children who develop the disease as infants are still alive five years after treatment, as are 35% of children whose disease developed later.

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