Neuroblastoma – Childhood

Overview

Neuroblastoma is a solid cancerous tumor that begins in the nerve cells of infants and young children. Neuroblastoma can start in nerve tissue of the neck, chest, abdomen, or pelvis, but it most often begins in the tissue of the adrenal glands. The adrenal glands are located on top of both kidneys and produce hormones that help control body functions, such as heart rate and blood pressure.

Neuroblasts are immature nerve cells found in unborn babies. Normal neuroblasts mature into nerve cells or adrenal medulla cells (cells found in the center of the adrenal gland). Neuroblastoma forms when neuroblasts don’t mature properly.

Sometimes, babies are born with small masses of neuroblasts that eventually mature into nerve cells and do not become cancer. A neuroblast that does not mature can continue to grow, forming a mass called a tumor.
Neuroblastoma develops most often in infants and children younger than five years old. It can form before the baby is born and can sometimes be found during a prenatal (before birth) ultrasound examination. Most often, however, neuroblastoma is found after the cancer has spread to other parts of the body, such as the lymph nodes (tiny, bean-shaped organs that help fight infection), liver, lungs, bones, and bone marrow (the spongy, red tissue in the inner part of large bones)

Risk Factors

For most types of cancer, a risk factor is anything that increases a person’s chance of developing cancer. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do.

Researchers have found inherited mutated (changed) genes that play a role in the development of neuroblastoma for children with a family history of the disease. However, the cause of neuroblastoma in children without a family history is not known. Neuroblastoma occurs more often in boys than in girls. Occasionally, a child who has one or more family members with the disease may also be diagnosed with neuroblastoma.

Family history and genetic predisposition

Approximately 1-2% of children with neuroblastoma have a family history of the disease. Children with an inherited predisposition tend to develop the disease, on average, nine to 13 months earlier than other children with neuroblastoma. In children who have a family history of neuroblastoma, the disease may occur in two or more organs.

Symptoms

Children with neuroblastoma may experience the following symptoms. Sometimes, children with neuroblastoma do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom on this list, please talk with your child’s doctor.
Many symptoms of neuroblastoma are caused by pressure from the tumor or bone pain if the cancer has spread to the bones. Pain may cause the child to limp, refuse to walk, or become unable to walk. Other symptoms include:

• A lump or mass in the abdomen, chest, neck, or pelvis, often found by a parent when bathing the child
• Skin lesions or nodules under the skin with blue or purple patches
• Protruding eyes and dark circles under the eyes (if the cancer has spread behind the eyes)
• Changes in the eyes, such as black eyes, a droopy eyelid, a pupil that is constricted, vision problems, or changes in the color of the iris

Treatment

In general, cancer in children is uncommon, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That’s why more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare the best treatments available (standard treatments) with newer treatments that may be more effective. Investigating new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track progress.

Surgery

A surgical oncologist is a doctor who specializes in treating cancer using surgery. If the tumor has not spread, surgery can sometimes be used to remove the entire tumor. However, most neuroblastoma is not found until after the cancer has spread. In that case, the doctor removes as much of the tumor as possible during surgery. If the tumor cannot be completely removed, the child may receive radiation therapy or chemotherapy (see below) to destroy the remaining cancer.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. Some people may receive chemotherapy in their doctor’s office; others may go to the hospital. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a specific time.