Neuroblastoma: Read About the Prognosis

Neuroblastoma facts*

*Neuroblastoma facts medical author: John P. Cunha, DO, FACOEP

Neuroblastoma is a disease in which cancer cells form in nerve tissue of the adrenal gland, neck, chest, or spinal cord.
Neuroblastoma is the third most common childhood cancer after leukemias and cancer of the central nervous system.
Common symptoms of neuroblastoma include a lump in the abdomen, neck, or chest; bulging eyes; dark circles around the eyes; bone pain; weakness or paralysis of a body part; and in infants — swollen stomach and bluish lumps under the skin.
To diagnose neuroblastoma, a doctor will take a history and perform a physical exam. They may also do a urine test, blood chemistry studies, X-rays, CT scans, neurological exams, ultrasound, and other tests, or take samples of bone marrow for analysis or biopsy. Many of these same tests can help determine the stage or extent of the neuroblastoma.
The prognosis for neuroblastoma depends on the age of the child when diagnosed, the stage of the cancer, where the tumor is located, and how quickly the tumor cells are growing.
There are four stages of neuroblastoma. Stages depend on whether the tumor can be completely removed surgically, if it has spread to lymph nodes or other parts of the body.
For neuroblastoma, treatment is based on risk groups: low risk, intermediate risk, and high risk. The stage of neuroblastoma is one factor used to determine risk group. Other factors are the age of the child, the tumor’s appearance under the microscope. its histology, and other measures of the tumor’s biology.
Standard treatment options include surgery, radiation therapy, chemotherapy, and watching and waiting to see how the disease progresses. Newer types of treatment include monoclonal antibody therapy, high-dose chemotherapy and radiation therapy with stem cell transplant, and other drug therapies. Taking part in clinical trials may be considered.

What is neuroblastoma?

Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue of the adrenal gland, neck, chest, or spinal cord.

Neuroblastoma often begins in the nerve tissue of the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. The adrenal glands produce important hormones that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Neuroblastoma may also begin in the chest, in nerve tissue near the spine in the neck, or in the spinal cord.

Neuroblastoma most often begins during early childhood, usually in children younger than 5 years. It sometimes forms before birth but is usually found later, when the tumor begins to grow and cause symptoms. In rare cases, neuroblastoma may be found before birth by fetal ultrasound.

By the time neuroblastoma is diagnosed, the cancer has usually metastasized (spread), most often to the lymph nodes, bones, bone marrow, liver, and skin.

Possible signs of neuroblastoma include bone pain and a lump in the abdomen, neck, or chest.

The most common symptoms of neuroblastoma are caused by the tumor pressing on nearby tissues as it grows or by cancer spreading to the bone. These and other symptoms may be caused by neuroblastoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

Lump in the abdomen, neck, or chest.
Bulging eyes.
Dark circles around the eyes ("black eyes").
Pains in the bones.
Swollen stomach and trouble breathing in infants.
Painless, bluish lumps under the skin in infants.
Weakness or paralysis (loss of ability to move a body part).

Less common signs of neuroblastoma include the following:

Fever.
Shortness of breath.
Feeling tired.
Easy bruising or bleeding.
Petechiae (flat, pinpoint, dark spots under the skin caused by bleeding).
High blood pressure.
Severe watery diarrhea.
Jerky muscle movements.
Uncontrolled eye movement.
Swelling of the legs, ankles, feet, or scrotum.

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Cancer is the result of the uncontrolled growth of abnormal cells anywhere in the body.
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Tests that examine many different body tissues and fluids are used to detect (find) and diagnose neuroblastoma.

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The following tests and procedures may be used:

Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of certain substances. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. A higher than normal amount of the substances homovanillic acid (HMA) and vanillyl mandelic acid (VMA) may be a sign of neuroblastoma.
Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. A higher than normal amount of the hormones dopamine and norepinephrine may be a sign of neuroblastoma.
Cytogenetic analysis: A test in which cells in a sample of blood or bone marrow are viewed under a microscope to look for certain changes in the chromosomes.
Bone marrow aspiration and biopsy: The removal of a small piece of bone, bone marrow, and blood by inserting a needle into the hipbone or breastbone. A pathologist views both the bone and the bone marrow samples under a microscope to look for signs of cancer. Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.
X-ray: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
Immunohistochemistry study: A procedure in which dyes or enzymes are added to a blood or bone marrow sample to test for certain antigens (proteins that stimulate the body’s immune response).

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Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

Age of the child when diagnosed. Stage of the cancer. Where the tumor is in the body. Tumor histology (the shape, function, and structure of the tumor cells). Prognosis and treatment decisions for neuroblastoma are also affected by tumor biology, which includes:

The patterns of the tumor cells. How different the tumor cells are from normal cells. How fast the tumor cells are growing. The number of chromosomes in the tumor cells. How many copies of the N-myc gene there are. The tumor biology is said to be favorable or unfavorable, depending on these factors. A favorable tumor biology means there is a better chance of recovery.

Stages of neuroblastoma

After neuroblastoma has been diagnosed, tests are done to find out if cancer has spread from where it started to other parts of the body.

The process used to find out the extent or spread of cancer is called staging. The information gathered from the staging process helps determine the stage of the disease. For neuroblastoma, stage is one of the factors used to plan treatment. The following tests and procedures may be used to determine the stage:

Bone marrow aspiration and biopsy: The removal of a small piece of bone, bone marrow, and blood by inserting a needle into the hipbone or breastbone. A pathologist views both the bone and bone marrow samples under a microscope to look for signs of cancer.
Lymph node biopsy: The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. One of the following types of biopsies may be done:

Excisional biopsy: The removal of an entire lymph node.
Incisional biopsy or core biopsy: The removal of part of a lymph node using a wide needle.
Needle biopsy or fine-needle aspiration: The removal of a sample of tissue or fluid from a lymph node using a thin needle.

CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
X-rays of the chest, bones, and abdomen: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
Radionuclide scan: A procedure to find areas in the body where cells, such as cancer cells, are dividing rapidly. A very small amount of radioactive material is swallowed or injected into a vein and travels through the bloodstream. The radioactive material collects in the bones or other tissues and is detected by a radiation-measuring device. These tests include bone scans, PET and MIBG scans.

The following stages are used for neuroblastoma:

Stage 1

In stage 1, the tumor is in only one area and all of the tumor that can be seen is completely removed during surgery.

Stage 2

Stage 2 is divided into stage 2A and 2B.

Stage 2A: The tumor is in only one area and all of the tumor that can be seen cannot be completely removed during surgery.
Stage 2B: The tumor is in only one area and all of the tumor that can be seen may be completely removed during surgery. Cancer cells are found in the lymph nodes near the tumor.

Stage 3

In stage 3, one of the following is true:

the tumor cannot be completely removed during surgery and has spread from one side of the body to the other side and may also have spread to nearby lymph nodes; or
the tumor is in only one area, on one side of the body, but has spread to lymph nodes on the other side of the body; or
the tumor is in the middle of the body and has spread to tissues or lymph nodes on both sides of the body, and the tumor cannot be removed by surgery.

Stage 4

Stage 4 is divided into stage 4 and stage 4S.

In stage 4, the tumor has spread to distant lymph nodes, the skin, or other parts of the body.
In stage 4S, the following are true:

the child is younger than 1 year; and
the cancer has spread to the skin, liver, and/or bone marrow, but not into the bone itself; and
the primary tumor is in only one area and all of the tumor in that location that can be seen may be completely removed during surgery; and/or
cancer cells may be found in the lymph nodes near the primary tumor.

Treatment of neuroblastoma is based on risk groups.

For many types of cancer, stages are used to plan treatment. For neuroblastoma, treatment depends on risk groups. The stage of neuroblastoma is one factor used to determine risk group. Other factors are the age of the child, tumor histology, and tumor biology.

There are 3 risk groups: low risk, intermediate risk, and high risk.

Low-risk and intermediate-risk neuroblastoma have a good chance of being cured.
High-risk neuroblastoma may be difficult to cure.

Progressive/recurrent neuroblastoma

Progressive neuroblastoma is cancer that has progressed (continued to grow) during treatment. Recurrent neuroblastoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the same place or in other parts of the body.

Treatment option overview

There are different types of treatment for patients with neuroblastoma.

Different types of treatment are available for patients with neuroblastoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Because cancer in children is rare, taking part in a clinical trial should be considered. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site 2. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

Children with neuroblastoma should always have their treatment planned by a team of doctors with expertise in treating childhood cancer.

Your child’s treatment will be managed by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist may refer you to other pediatric doctors who have experience and expertise in treating children with neuroblastoma and who specialize in certain areas of medicine. These may include the following specialists:

Medical oncologist.
Hematologist.
Pediatric surgeon.
Radiation oncologist.
Endocrinologist.
Neurologist.
Neuropathologist.
Neuroradiologist.
Pediatric nurse specialist.
Social worker.
Rehabilitation specialist.
Psychologist.

Children who are treated for neuroblastoma may be at higher risk for second cancers.

Some cancer treatments cause side effects that continue or appear years after cancer treatment has ended. These are called late effects. Late effects of cancer treatment include physical problems; changes in mood, feelings, actions, thinking, learning, or memory; and second cancers.

Some late effects may be treated or controlled. It is important that parents of children who are treated for neuroblastoma talk with their doctors about the possible late effects caused by some treatments.

Four types of standard treatment are used:

Surgery

Surgery is usually used to treat neuroblastoma. Depending on where the tumor is and whether it has spread, as much of the tumor as possible will be removed. If the tumor cannot be removed, a biopsy may be done instead.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

The use of two or more anticancer drugs is called combination chemotherapy.

Watchful waiting

Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change.

New types of treatment are being tested in clinical trials. These include the following:

Monoclonal antibody therapy

Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to deliver drugs, toxins, or radioactive material directly to cancer cells.

High-dose chemotherapy and radiation therapy with stem cell transplant

High-dose chemotherapy and radiation therapy with stem cell transplant is a way of giving high doses of chemotherapy and radiation therapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy and radiation therapy are completed, the stored stem cells are thawed and given back to the patient through an infusion, just like a blood transfusion. These reinfused stem cells migrate to the bone marrow and grow into (and restore) the body’s bone marrow so it can again make blood cells.

Other drug therapy

13-cis retinoic acid is a vitamin -like drug that slows the cancer’s ability to make more cancer cells and changes how these cells look and act.

This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.

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Treatment options for neuroblastoma

Low-risk neuroblastoma

Treatment of low-risk neuroblastoma may include the following:

Surgery followed by watchful waiting.
Watchful waiting alone for certain infants.
Surgery followed by chemotherapy, when less than half of the tumor is removed or when serious symptoms cannot be relieved by surgery.
Radiation therapy to treat tumors that are causing serious problems and do not respond quickly to chemotherapy.
Low-dose chemotherapy.

Intermediate-risk neuroblastoma

Treatment of intermediate-risk neuroblastoma may include the following:

Chemotherapy.
Chemotherapy followed by surgery and/or radiation therapy.
Radiation therapy to treat tumors that are causing serious problems and do not respond quickly to chemotherapy.

High-risk neuroblastoma

Treatment of high-risk neuroblastoma may include the following:

High-dose chemotherapy followed by surgery to remove as much of the tumor as possible.
Radiation therapy to the tumor site and, if needed, to other parts of the body with cancer.
Stem cell transplant.
Chemotherapy followed by 13-cis retinoic acid.
A clinical trial of monoclonal antibody therapy after chemotherapy.
A clinical trial of radiation therapy with radioactive iodine before stem cell transplant.
A clinical trial of stem cell transplant followed by 13-cis retinoic acid.

This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied.

Progressive/recurrent neuroblastoma

Patients first treated for low-risk neuroblastoma

Treatment for recurrent neuroblastoma that is found in one place in the body may include the following:

Surgery followed by watchful waiting or chemotherapy.
Chemotherapy.
High-dose chemotherapy, stem cell transplant, and 13-cis retinoic acid.

Treatment for recurrent neuroblastoma that has spread to other parts of the body may include the following:

Watchful waiting.
Surgery followed by chemotherapy.
Chemotherapy.
High-dose chemotherapy, stem cell transplant, and 13-cis retinoic acid.
A clinical trial of a new treatment.

Patients first treated for intermediate-risk neuroblastoma

For recurrent neuroblastoma that is found in one place in the body, treatment is usually surgery, with or without chemotherapy.

For recurrent neuroblastoma that has spread to other parts of the body, treatment is usually high-dose chemotherapy, stem cell transplant, and 13-cis retinoic acid.

Patients first treated for high-risk neuroblastoma

Treatment of recurrent neuroblastoma in patients first treated for high-risk neuroblastoma may include the following:

A clinical trial of chemotherapy followed by monoclonal antibody therapy.
A clinical trial of radiation therapy with radioactive iodine, alone or before stem cell transplant.
A clinical trial of stem cell transplant.

This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied.

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