About neuroblastoma

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 (" 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.

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).

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.

What are the symptoms for neuroblastoma?

Lump in abdomen symptom was found in the neuroblastoma condition

Signs and symptoms of neuroblastoma vary depending on what part of the body is affected.

Neuroblastoma in the abdomen — the most common form — may cause signs and symptoms such as:

  • Abdominal pain
  • A mass under the skin that isn't tender when touched
  • Changes in bowel habits, such as Diarrhea or constipation

Neuroblastoma in the chest may cause signs and symptoms such as:

  • Wheezing
  • Chest pain
  • Changes to the eyes, including drooping eyelids and unequal pupil size

Other signs and symptoms that may indicate neuroblastoma include:

  • Lumps of tissue under the skin
  • Eyeballs that seem to protrude from the sockets (proptosis)
  • Dark circles, similar to bruises, around the eyes
  • Back pain
  • Fever
  • Unexplained weight loss
  • Bone pain

When to see a doctor

Contact your child's doctor if your child has any signs or symptoms that worry you. Mention any changes in your child's behavior or habits.

What are the causes for neuroblastoma?

In general, cancer begins with a genetic mutation that allows normal, healthy cells to continue growing without responding to the signals to stop, which normal cells do. Cancer cells grow and multiply out of control. The accumulating abnormal cells form a mass (tumor).

Neuroblastoma begins in neuroblasts — immature nerve cells that a fetus makes as part of its development process.

As the fetus matures, neuroblasts eventually turn into nerve cells and fibers and the cells that make up the adrenal glands. Most neuroblasts mature by birth, though a small number of immature neuroblasts can be found in newborns. In most cases, these neuroblasts mature or disappear. Others, however, form a tumor — a neuroblastoma.

It isn't clear what causes the initial genetic mutation that leads to neuroblastoma.

What are the treatments for neuroblastoma?

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.

What are the risk factors for neuroblastoma?

Children with a family history of neuroblastoma may be more likely to develop the disease. Yet, familial neuroblastoma is thought to comprise a very small number of neuroblastoma cases. In most cases of neuroblastoma, a cause is never identified.

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