The body’s nervous system is divided into two main areas: the central nervous system (which includes the brain and the spinal cord) and the peripheral nervous system. The sympathetic nervous system, part of the peripheral nervous system, controls the “fight or flight” response, the levels of certain hormones, digestion, heart rate, and blood pressure. It helps carry messages from your brain to various parts of your body.
Neuroblastoma is a cancer that develops in tissues of the sympathetic nervous system. It is a rare cancer and according to Memorial Sloan-Kettering Cancer Center (MSKCC, 2012). there are approximately 700 new cases diagnosed each year in the United States.
It is typically diagnosed before a person reaches the age of 5, and is the most common cancer in infants.
It is a solid tumor and is often found in the adrenal gland, pelvis, abdomen, neck, or chest. If it progresses, it can spread to the bones, lymph nodes, and skin.
Early symptoms of the cancer tend to be generalized and non-specific. They can include fever, loss of appetite, diarrhea, and weight loss. Remember that these symptoms do not necessarily point to a diagnosis of neuroblastoma.
Later signs and symptoms depend on the location of the tumor and can include:
- bone pain
- breathing difficulties
- swollen abdomen
- enlarged lymph nodes
- excessive sweating
- rapid heart rate
- high blood pressure
- trouble urinating
- involuntary, uncontrolled movements of eyes, feet and legs
- loss of movement of hips and lower extremities—even to the point of complete paralysis
- difficulties with body balance
Due to the nonspecific nature of the symptoms of early neuroblastoma, the disease tends to be extensive at the time of diagnosis. According to Memorial-Sloan Kettering, half of all cases have already spread by the time they are diagnosed (MSKCC, 2012).
Tests used to aid in the diagnosis are:
- blood tests
- urine tests
- bone marrow biopsy
- CT scan
- bone scan
- PET scan
Once neuroblastoma has been diagnosed, the disease is staged. This means it is categorized based on its location and how far it has spread. The stage of the cancer determines the treatment, which is why staging is crucial to the outcome. There are four stages of neuroblastoma:
The tumor is in one area and can be removed fairly easily.
In Stage 2A, the tumor is in one area but cannot be completely removed during surgery.
In Stage 2B, the tumor is in one area and CAN completely removed during surgery. However, in addition to the tumor that can be seen, cancer cells are found in the lymph nodes near the tumor.
If any of the following three situations are true, the neuroblastoma is defined as Stage 3.
- The tumor can’t be entirely removed during surgery. In addition, it has spread from one side of the body to the other side. It may or may not have spread to nearby lymph nodes.
- The tumor is still in the one area it started, and is only one side of the body. However, cancer cells have been found in lymph nodes on the other side of the body.
- The tumor is in the middle of the body, but it is spreading to towards both sides of the body. This is due either to growth of the tumor itself, or the spread of cancer cells through lymph nodes.
In this stage, the tumor and/or cancer cells have spread to distant parts of the body such as bone, liver, skin, distant lymph nodes or other organs.
Stage 4S is a special category of neuroblastoma that behaves differently. Stage IV neuroblastoma is when the following criteria are true:
- the child is younger than 1 year
- the cancer is on one side of the body (it may have spread to lymph nodes on this side, but has not spread to the other side)
- the tumor has spread to the child’s liver, skin, and/or bone marrow
- less than 10% of marrow cells are cancerous
- the cancer has NOT spread to the bones
Once staged, tumors are grouped into low, intermediate, or high risk. Risk groups are determined by the stage as well as the age of the patient and the tumor histology and biology. Low-risk and intermediate-risk neuroblastoma have a good chance of being fully cured. High-risk neuroblastoma may be difficult to cure.
Treatment for this cancer depends on the age of the child and the stage of the cancer. It can include surgery, chemotherapy, radiation, immunotherapy, or stem cell transplant. Many patients will have more than one type of treatment. Treatment is typically done in stages and can last several years.
Chemotherapy is the use of anti-cancer drugs to kill the disease. These drugs are usually given intravenously, but they can also be taken orally, depending on the specific drug. Side effects can include hair loss, mouth sores, nausea and vomiting, lowered immune system function, and fatigue. Side effects go away once treatment is finished.
Radiation uses high-energy rays like X-rays to kill cancer cells. It is typically administered by a machine that aims the rays at the desired area. This type of treatment can cause side effects like skin irritation, diarrhea, and fatigue.
Immunotherapy is also called biologic therapy. This treatment uses medications to stimulate the immune system to fight disease.
Stem Cell Therapy
Stem cell therapy is also referred to as bone marrow transplant. After you receive high doses of chemotherapy and/or radiation, replacement stem cells are injected into your bloodstream. This treatment is not used for everyone, and is typically for high-risk patients whose treatment options likely to result in cure are limited.
Each child is different, and your doctor will talk with you about the possible treatment options that are best for your child and your child’s proposed treatment schedule.
According to the Children’s Neuroblastoma Cancer Foundation, survival rate for high-risk cases is between 25 and 50 percent, and there is little chance for relapse for low- and medium-risk cases that have been successfully treated (CNCF, 2012). After treatment for neuroblastoma is over, follow-up appointments are necessary to monitor for any relapse of disease. Checkups are important because cancer treatment can cause long-term side effects. While not everyone who has neuroblastoma will experience serious side effects, they are a possibility. These serious effects can include learning difficulties, vision problems, seizures, developmental problems, muscle and bone issues, and secondary cancers.
Talk with your doctor about the risks of the specific treatments and how future complications can be monitored, treated, or avoided.
Many cancer centers and hospitals have support groups for patients and families that are dealing with or have dealt with neuroblastoma or other cancers. You may find these helpful for support and information.