Previously Untreated Childhood Rhabdomyosarcoma
        Surgery options
        Chemotherapy options
        Radiation therapy options
Recurrent Childhood Rhabdomyosarcoma

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Childhood rhabdomyosarcoma may be treated with chemotherapy and/or radiation therapy before or after surgery. Treatment with surgery may include the following:

Rhabdomyosarcoma of the head and neck

• For tumors of the head and neck that are close to the skin and not in or near the eye: Treatment may include surgery (wide local excision). Some lymph nodes may be removed from the same side of the neck as the tumor. Surgery will be followed by chemotherapy with or without radiation therapy.
• For tumors of the head and neck that are in or near the eye: A biopsy of the tumor is followed by chemotherapy and radiation therapy. If the tumor remains or comes back after treatment with chemotherapy and radiation therapy, surgery to remove the eye and some tissues around the eye may be needed.
• For tumors of the head and neck that cannot be removed by surgery: Chemotherapy and radiation therapy are given.

Rhabdomyosarcoma of the arms or legs

• For tumors of the arms or legs: Surgery (wide local excision and en bloc removal of a cuff of normal tissue) may be done. For tumors in the arms, lymph nodes near the tumor and in the armpit area may be removed. For tumors in the legs, lymph nodes near the tumor and in the groin area may be removed. A second surgery may be done to remove any remaining tumor cells.

Rhabdomyosarcoma of the chest or abdomen

• For tumors in the chest wall or abdominal wall: Surgery (wide local excision) may be done. A second surgery may be done to remove any remaining tumor cells.
• For tumors of the chest or abdomen: Surgery alone or chemotherapy and radiation therapy, followed by surgery to remove the tumor.
• For large tumors of the chest or abdomen: Chemotherapy and/or radiation therapy is given to shrink the tumor first, followed by surgery (wide local excision) to remove the remaining tumor.
• For tumors of the muscles or tissues around the anus or between the vulva and the anus or the scrotum and the anus: Surgery is done to remove as much of the tumor as possible and some nearby lymph nodes, followed by chemotherapy.
• For tumors of the gallbladder or bile ducts: Surgery is done to remove as much of the tumor as possible, followed by chemotherapy.

Rhabdomyosarcoma of the area near the testicles

• Rhabdomyosarcoma of the testicular area is usually treated with surgery to remove the testicle and spermatic cord. Sometimes the lymph nodes in the back of the abdomen are removed, especially if the child is older than 9 years. CT scans may be done every 3 months after surgery to see if the cancer is growing in nearby lymph nodes.

Rhabdomyosarcoma of the prostate or bladder

• For tumors that are only at the top of the bladder: Surgery (wide local excision) is done.
• For tumors of the prostate or other parts of the bladder: Chemotherapy and radiation therapy are given first to shrink the tumor. A biopsy is done and if cancer cells remain, surgery to remove the tumor is done. Surgery may include removal of the prostate, part of the bladder, or pelvic exenteration without removal of the rectum. (This may include removal of the lower colon and bladder. In girls, the cervix, vagina, ovaries, and nearby lymph nodes may be removed).

Rhabdomyosarcoma of the vagina, vulva, or uterus

• Rhabdomyosarcoma of the vagina, vulva, or uterus is usually treated with a biopsy followed by chemotherapy.
• For tumors of the vagina, vulva, or uterus: Chemotherapy is given first to shrink the tumor, followed by surgery. Radiation therapy may be given after surgery.

Rhabdomyosarcoma in unusual areas

• For tumors of the diaphragm: A biopsy of the tumor is followed by chemotherapy to shrink the tumor. Surgery may be done later to remove any remaining cancer cells.
• For tumors of the larynx (voice box): A biopsy of the tumor is followed by chemotherapy and radiation therapy. Surgery is usually not done, so that the voice can be saved.

Metastatic rhabdomyosarcoma

• Metastatic rhabdomyosarcoma is not usually treated with surgery. However, if the cancer has spread to the lungs, chemotherapy and radiation therapy are given to shrink the tumor, followed by surgery to remove any remaining cancer cells in the lung.

Every child treated for rhabdomyosarcoma should receive chemotherapy. The dose of the chemotherapy and the number of treatments given depend on the child's risk group ¹, as follows:

Low-risk patients

• Combination chemotherapy, with or without radiation therapy.
• A clinical trial of different doses and schedules of combination chemotherapy, with or without radiation therapy.

Intermediate-risk patients

• Combination chemotherapy.
• A clinical trial of combination chemotherapy with radiation therapy.

High-risk patients

• Combination chemotherapy.
• A clinical trial of different doses and schedules of combination chemotherapy, with or without radiation therapy.
• A clinical trial of high-dose chemotherapy with stem cell transplant for high-risk childhood rhabdomyosarcoma.

Radiation therapy may be used if childhood rhabdomyosarcoma tumor cells remain after surgery, after chemotherapy, or if the tumor is the alveolar type.

Treatment may include certain kinds of radiation therapy that cause less damage to normal tissue and lessen late effects of treatment. These include:

• Conformal radiation uses a computer to create a 3-dimensional picture of the tumor. The radiation beams are shaped to fit the tumor.
• Intensity-modulated radiation therapy (IMRT) uses images created by a computer that show the size and shape of the tumor. Thin beams of radiation of different intensities are aimed at the tumor from many angles.
• Proton-beam therapy is a type of high-energy, external radiation therapy that uses streams of protons (small, positively-charged particles of matter) to produce radiation.
• A clinical trial of brachytherapy (also called internal radiation therapy) for cancer in areas such as the vagina, vulva, head, or neck.
• A clinical trial of second-look surgery after chemotherapy to reduce the need for radiation therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with previously untreated childhood rhabdomyosarcoma ².

Treatment options for recurrent childhood rhabdomyosarcoma are based on many factors, including where in the body the cancer has come back, what type of treatment the patient had before, and the needs of the individual child. Treatment may include one or more of the following:

• Chemotherapy with one or more anticancer agents.
• Surgery.
• Radiation therapy.
• A clinical trial of different schedules and combinations of chemotherapy.
• A clinical trial of new chemotherapy regimens.
• A clinical trial of new anticancer drugs.
• A clinical trial of high-dose chemotherapy followed by stem cell transplant using the patient's own stem cells.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood rhabdomyosarcoma ³.

Glossary Terms

3-D. A graphic display of depth, width, and height. Also called 3-D.

A procedure that uses a computer to create a 3-dimensional picture of the tumor. This allows doctors to give the highest possible dose of radiation to the tumor, while sparing the normal tissue as much as possible. Also called 3-dimensional radiation therapy.

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

Having to do with the abdomen, which is the part of the body between the chest and the hips that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

The opening of the rectum to the outside of the body.

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

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.

The organ that stores urine.

A type of radiation therapy in which radioactive material sealed in needles, seeds, wires, or catheters is placed directly into or near a tumor. Also called radiation brachytherapy, internal radiation therapy, and implant radiation therapy.

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

The lower, narrow end of the uterus that forms a canal between the uterus and vagina.

Treatment with drugs that kill cancer cells.

The muscles, bones, and joints that make up the area of the body between the neck and the abdomen.

A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called a clinical study.

The longest part of the large intestine, which is a tube-like organ connected to the small intestine at one end and the anus at the other. The colon removes water and some nutrients and electrolytes from partially digested food. The remaining material, solid waste called stool, moves through the colon to the rectum and leaves the body through the anus.

Treatment using more than one anticancer drug.

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.

The thin muscle below the lungs and heart that separates the chest from the abdomen.

The amount of medicine taken, or radiation given, at one time.

Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.

A type of radiation therapy that uses a machine to aim high-energy rays at the cancer from outside of the body. Also called external-beam radiation therapy.

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

The area where the thigh meets the abdomen.

An intensive drug treatment to kill cancer cells, but that also destroys the bone marrow and can cause other severe side effects. High-dose chemotherapy is usually followed by bone marrow or stem cell transplantation to rebuild the bone marrow.

IMRT. A type of 3-dimensional radiation therapy that uses computer-generated images to show the size and shape of the tumor. Thin beams of radiation of different intensities are aimed at the tumor from many angles. This type of radiation therapy reduces the damage to healthy tissue near the tumor. Also called IMRT.

The area of the throat containing the vocal cords and used for breathing, swallowing, and talking. Also called voice box.

Side effects of cancer treatment that appear months or years after treatment has ended. Late effects include physical and mental problems and second cancers.

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

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.

Having to do with metastasis, which is the spread of cancer from one part of the body to another.

One of a pair of female reproductive glands in which the ova, or eggs, are formed. The ovaries are located in the pelvis, one on each side of the uterus.

Surgery to remove the lower colon, rectum, and bladder, and create stomata (openings) through which urine and stool are passed out of the body. In women, the cervix, vagina, ovaries, and nearby lymph nodes are also removed.

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.

A small, positively charged particle of matter found in the atoms of all elements. Streams of protons generated by special equipment can be used for radiation treatment.

A type of high-energy, external radiation therapy that uses streams of protons (small, positively charged particles) that come from a special machine. Proton beam radiation is different from x-ray radiation.

Energy released in the form of particles or electromagnetic waves. Common sources of radiation include radon gas, cosmic rays from outer space, and medical x-rays.

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called radiotherapy and irradiation.

The last several inches of the large intestine. The rectum ends at the anus.

Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.

A treatment plan that specifies the dosage, the schedule, and the duration of treatment.

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

In clinical trials, the step-by-step plan for how patients are to be treated; for example, the drug or type of radiation therapy that is to be given, the method by which it is to be given, the amount of time between courses, and the total length of treatment.

In males, the external sac that contains the testicles.

Surgery performed after primary treatment to determine whether tumor cells remain.

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.

A cell from which other types of cells develop. For example, blood cells develop from blood-forming stem cells.

A method of replacing immature blood-forming cells that were destroyed by cancer treatment. The stem cells are given to the person after treatment to help the bone marrow recover and continue producing healthy blood cells.

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 found inside the scrotum that produce sperm and male hormones. Also called a testis.

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 small, hollow, pear-shaped organ in a woman's pelvis. This is the organ in which a baby grows. Also called the womb.

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

The external female genital organs, including the clitoris, vaginal lips, and the opening to the vagina.

Surgery to cut out the cancer and some healthy tissue around it.