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.

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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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.