Key Points for This Section

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. There are three ways that cancer spreads in the body. The following stages are used for neuroblastoma: Stage 1 Stage 2 Stage 3 Stage 4 Treatment of neuroblastoma is based on risk groups.

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 bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone 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: The removal of part of a lymph node.
  • Core biopsy: The removal of tissue from a lymph node using a wide needle.
  • Fine-needle aspiration (FNA) biopsy: The removal 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. The picture can be printed to be looked at later.
• 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.

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

• Through tissue. Cancer invades the surrounding normal tissue.
• Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
• Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.

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; and
  • the tumor is in only one area and all of the tumor that can be seen may be completely removed during surgery; and/or
  • cancer cells may be found in the lymph nodes near the 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.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

Primary bone cancer is cancer that forms in cells of the bone. Some types of primary bone cancer are osteosarcoma, Ewing sarcoma, malignant fibrous histiocytoma, and chondrosarcoma. Secondary bone cancer is cancer that spreads to the bone from another part of the body (such as the prostate, breast, or lung).

The soft, sponge-like tissue in the center of most bones. It produces white blood cells, red blood cells, and platelets.

The removal of a small sample of bone marrow (usually from the hip) through a needle for examination under a microscope.

The removal of a sample of tissue from the bone marrow with a needle for examination under a microscope.

Cancer that forms in tissues of the breast, usually the ducts (tubes that carry milk to the nipple) and lobules (glands that make milk). It occurs in both men and women, although male breast cancer is rare.

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord.

The smallest type of blood vessel. A capillary connects an arteriole (small artery) to a venule (small vein) to form a network of blood vessels in almost all parts of the body. The wall of a capillary is thin and leaky, and capillaries are involved in the exchange of fluids and gases between tissues and the blood.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.

The removal of a tissue sample with a wide needle for examination under a microscope.

Computed tomography scan. A series of detailed pictures of areas inside the body taken from different angles; the pictures are created by a computer linked to an x-ray machine. Also called computed tomography scan, computerized tomography, computerized axial tomography scan, and CAT scan.

To heal or restore health; a treatment to restore health.

A surgical procedure in which an entire lump or suspicious area is removed for diagnosis. The tissue is then examined under a microscope.

The removal of tissue or fluid with a thin needle for examination under a microscope. Also called FNA biopsy.

Liquid.

The study of tissues and cells under a microscope.

A surgical procedure in which a portion of a lump or suspicious area is removed for diagnosis. The tissue is then examined under a microscope.

Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."

Cancer that has spread beyond the layer of tissue in which it developed and is growing into surrounding, healthy tissues. Also called infiltrating cancer.

A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.

The clear fluid that travels through the lymphatic system and carries cells that help fight infections and other diseases. Also called lymphatic fluid.

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.

A thin tube that carries lymph (lymphatic fluid) and white blood cells through the lymphatic system. Also called lymphatic vessel.

The tissues and organs that produce, store, and carry white blood cells that fight infections and other diseases. This system includes the bone marrow, spleen, thymus, lymph nodes, and lymphatic vessels (a network of thin tubes that carry lymph and white blood cells). Lymphatic vessels branch, like blood vessels, into all the tissues of the body.

The spread of cancer from one part of the body to another. A tumor formed by cells that have spread is called a “metastatic tumor” or a “metastasis.” The metastatic tumor contains cells that are like those in the original (primary) tumor. The plural form of metastasis is metastases (meh-TAS-tuh-SEEZ).

A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, nuclear magnetic resonance imaging, and NMRI.

Cancer that arises in immature nerve cells and affects mostly infants and children.

A part of the body that performs a specific function. For example, the heart is an organ.

A doctor who identifies diseases by studying cells and tissues under a microscope.

The original tumor.

Energy released in the form of particle or electromagnetic waves. Common sources of radiation include radon gas, cosmic rays from outer space, medical x-rays, and energy given off by a radioisotope (unstable form of a chemical element that releases radiation as it breaks down and becomes more stable).

Giving off radiation.

A procedure that produces pictures (scans) of structures inside the body, including areas where there are cancer cells. Radionuclide scanning is used to diagnose, stage, and monitor disease. A small amount of a radioactive chemical (radionuclide) is injected into a vein or swallowed. Different radionuclides travel through the blood to different organs. A machine with a special camera moves over the person lying on a table and detects the type of radiation given off by the radionuclides. A computer forms an image of the areas where the radionuclide builds up. These areas may contain cancer cells. Also called scintigraphy.

A computer picture of areas inside the body created by bouncing high-energy sound waves (ultrasound) off internal tissues or organs. Also called an ultrasonogram.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

Tumor is only found in one area; all of the tumor that can be seen is completely removed during surgery.

Stage 2 is divided into stages 2A and 2B. In stage 2A, the tumor is in one area only and not all of the tumor that can be seen can be completely removed during surgery. In stage 2B, the tumor is in one area only and all of the tumor that can be seen may be completely removed during surgery, but cancer cells are found in nearby lymph nodes.

In stage 3 neuroblastoma, the tumor (1) 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 (2) is in one area of one side of the body only, but has spread to lymph nodes on the other side of the body; or (3) 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 neuroblastoma is divided into stages 4 and 4S. In stage 4, the tumor has spread to distant lymph nodes, the skin, and/or other parts of the body. In stage 4S, (1) the child is younger than 1 year; and (2) the cancer has spread to the skin, liver, and/or bone marrow; and (3) the tumor is in one area only and all of the tumor that can be seen may be completely removed during surgery; and/or (4) cancer cells may be found in the lymph nodes near the tumor.

Performing exams and tests to learn the extent of the cancer within the body, especially whether the disease has spread from the original site to other parts of the body. It is important to know the stage of the disease in order to plan the best treatment.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

A group or layer of cells that work together to perform a specific function.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancerous), or malignant (cancerous). Also called neoplasm.

A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echo patterns are shown on the screen of an ultrasound machine, forming a picture of body tissues called a sonogram. Also called ultrasonography.

A blood vessel that carries blood to the heart from tissues and organs in the body.

A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.