Key Points for This Section

After childhood rhabdomyosarcoma has been diagnosed, treatment is based on the stage of the cancer and whether cancer remains after surgery to remove the tumor. There are three ways that cancer spreads in the body. Staging of childhood rhabdomyosarcoma is done in three related steps. The grouping system is based on whether the cancer has spread and how much cancer remains after surgery to remove the tumor: Group I Group II Group III Group IV The staging system is based on tumor location and size, and whether it has spread to lymph nodes or distant parts of the body: Stage 1 Stage 2 Stage 3 Stage 4 The risk group is based on both the grouping system and the staging system and is used to plan treatment. Low-risk childhood rhabdomyosarcoma Intermediate-risk childhood rhabdomyosarcoma High-risk childhood rhabdomyosarcoma

After childhood rhabdomyosarcoma has been diagnosed, treatment is based on the stage of the cancer and whether cancer remains after surgery to remove the tumor.

The process used to find out if cancer has spread within the muscle or to other parts of the body is called staging. It is important to know the stage in order to plan treatment. The doctor will use results of the diagnostic tests ¹ to help determine the stage of the disease.

Treatment for childhood rhabdomyosarcoma is based on the stage and the amount of cancer that remains after surgery to remove the tumor. The pathologist will use a microscope to check the tissues, including lymph nodes, removed during surgery, and the edges of the areas where the cancer was removed. This is done to see if all the cancer cells were taken out during the surgery.

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.

Staging of childhood rhabdomyosarcoma is done in three related steps.

Childhood rhabdomyosarcoma is staged by using three different ways to describe the cancer:

• A grouping system.
• A staging system.
• A risk group.

The grouping system is based on whether the cancer has spread and how much cancer remains after surgery to remove the tumor:

Group I

Cancer was only in the place where it started and it was completely removed by surgery. No cancer cells are seen when tissue taken from the edges of where the tumor was removed is viewed under a microscope by a pathologist.

Group II

Group II is divided into groups IIA, IIB, and IIC.

• IIA: Cancer was removed by surgery but there are cancer cells remaining that can be seen only with a microscope.
• IIB: Cancer had spread to nearby lymph nodes and the cancer and lymph nodes were removed by surgery.
• IIC: Cancer had spread to nearby lymph nodes and the cancer and lymph nodes were removed by surgery. There are cancer cells remaining that can be seen only with a microscope.

Group III

Cancer was removed by surgery and there are cancer cells remaining that can be seen without a microscope. Cancer did not spread to distant parts of the body.

Group IV

Cancer had spread to distant parts of the body at the time of diagnosis.

The staging system is based on tumor location and size, and whether it has spread to lymph nodes or distant parts of the body:

Stage 1

In stage 1, cancer is any size, has not spread to lymph nodes, and is found in only one of the following "favorable" sites:

• Eye or area around the eye.
• Head and neck (but not in the tissue covering the brain and spinal cord).
• Gallbladder and bile ducts.
• Near the testes or vagina (but not in the kidney, bladder, or prostate).

Rhabdomyosarcoma that occurs in a "favorable" site has a better prognosis. If the site where cancer occurs is not one of the "favorable" sites listed above, it is considered an "unfavorable" site.

Enlarge

Pea, peanut, walnut, and lime show tumor sizes.

Stage 2

In stage 2, cancer is found in any one area not included in stage 1. The tumor is 5 centimeters or smaller and has not spread to lymph nodes.

Stage 3

In stage 3, cancer is found in any one area not included in stage 1 and one of the following is true:

• The tumor is 5 centimeters or smaller and cancer has spread to nearby lymph nodes.
• The tumor is larger than 5 centimeters and cancer may have spread to nearby lymph nodes.

Stage 4

In stage 4, the tumor may be any size and cancer may have spread to nearby lymph nodes. Cancer has spread to distant parts of the body such as the lung, bone marrow, or bone.

The risk group is based on both the grouping system and the staging system and is used to plan treatment.

The following risk groups are used:

Low-risk childhood rhabdomyosarcoma

Low-risk childhood rhabdomyosarcoma is one of the following:

• An embryonal tumor of any size that is found in a "favorable" site. There may be tumor remaining after surgery that can be seen without a microscope. The cancer may have spread to lymph nodes. The following areas are "favorable" sites:
  • Eye or area around the eye.
  • Head and neck (but not in the tissue covering the brain and spinal cord).
  • Gallbladder and bile ducts.
  • Near the testes or vagina (but not in the kidney, bladder, or prostate).
• An embryonal tumor of any size that is not found in one of the "favorable" sites listed above. There may be tumor remaining after surgery that can be seen only with a microscope. The cancer may have spread to lymph nodes.

Intermediate-risk childhood rhabdomyosarcoma

Intermediate-risk childhood rhabdomyosarcoma is one of the following:

• An embryonal tumor of any size that is not found in one of the "favorable" sites listed above. There is tumor remaining after surgery, that can be seen with or without a microscope. The cancer may have spread to nearby lymph nodes.
• An alveolar tumor of any size in a "favorable" or "unfavorable" site. There may be tumor remaining after surgery that can be seen with or without a microscope. The cancer may have spread to nearby lymph nodes.

High-risk childhood rhabdomyosarcoma

High-risk childhood rhabdomyosarcoma may be the embryonal type, alveolar type, or anaplastic type. It may have spread to nearby lymph nodes and has spread to distant parts of the body.

Glossary Terms

A term used to describe cancer cells that divide rapidly and have little or no resemblance to normal cells.

A tube through which bile passes in and out of the liver.

The organ that stores urine.

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.

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 measure of length in the metric system. There are 100 centimeters in a meter and 2½ centimeters in an inch.

The process of identifying a disease, such as cancer, from its signs and symptoms.

Refers to cancer that has spread from the original (primary) tumor to distant organs or distant lymph nodes. Also known as distant cancer.

A soft-tissue tumor that affects children. It begins in muscle cells, and usually occurs in the head, neck, arms, legs, or genitourinary tract.

The pear-shaped organ found below the liver. Bile is concentrated and stored in the gallbladder.

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

One of a pair of organs in the abdomen. Kidneys remove waste from the blood (as urine), produce erythropoietin (a substance that stimulates red blood cell production), and play a role in blood pressure regulation.

One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.

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

The original tumor.

The likely outcome or course of a disease; the chance of recovery or recurrence.

A gland in the male reproductive system. The prostate surrounds the part of the urethra (the tube that empties the bladder) just below the bladder, and produces a fluid that forms part of the semen.

Cancer that forms in the soft tissues in a type of muscle called striated muscle. Rhabdomyosarcoma can occur anywhere in the body.

A column of nerve tissue that runs from the base of the skull down the back. It is surrounded by three protective membranes, and is enclosed within the vertebrae (back bones). The spinal cord and the brain make up the central nervous system, and spinal cord nerves carry most messages between the brain and the rest of the body.

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.

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.

One of two egg-shaped glands inside the scrotum that produce sperm and male hormones. Also called testicle.

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.

The muscular canal extending from the uterus to the exterior of the body. Also called birth canal.

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