Tratamiento del rabdomiosarcoma
At the Montefiore Einstein Comprehensive Cancer Center at the Children’s Hospital at Montefiore Einstein (CHAM), you’ll find nationally renowned, comprehensive care for pediatric rhabdomyosarcoma, a type of cancer that forms in muscle tissue. We know that each child’s cancer is different, so we personalize and target treatment, making sure your child receives the therapies that will be safest and most effective for them.
We provide a detailed initial evaluation, including a biopsy by a pathologist who specializes in the diagnosis of sarcomas. This helps us determine the treatments most likely to be effective for your child and to evaluate whether they are candidates for clinical trials that test new treatment approaches. Physicians on CHAM’s pediatric sarcoma team lead many of the clinical trials across the country to improve treatments and find a cure. Through our research efforts, our team works to uncover the most advanced, up-to-date and promising new treatments.
Cuando desee lo mejor para su hijo, recurra a los especialistas del Montefiore Einstein Comprehensive Cancer Center en CHAM, quienes se apasionan por terminar con el cáncer y abordar todas las necesidades de salud de su hijo.
Como centro del cáncer designado por el Instituto Nacional del Cáncer (NCI, por sus siglas en inglés) Montefiore Einstein Comprehensive Cancer Center apoya la misión y las normas del NCI. La siguiente información sobre los tipos de cáncer, prevención y tratamientos ha sido facilitada por el NCI.
Childhood Rhabdomyosarcoma Treatment (PDQ®)–Patient Version
General Information About Childhood Rhabdomyosarcoma
Puntos clave
- Childhood rhabdomyosarcoma is a disease in which malignant (cancer) cells form in muscle tissue.
- Certain genetic conditions increase the risk of childhood rhabdomyosarcoma.
- A sign of childhood rhabdomyosarcoma is a lump or swelling that keeps getting bigger.
- Diagnostic tests and a biopsy are used to diagnose childhood rhabdomyosarcoma.
- Hay algunos factores que afectan al pronóstico (es decir, la posibilidad de recuperación) y a las opciones de tratamiento.
Childhood rhabdomyosarcoma is a disease in which malignant (cancer) cells form in muscle tissue.
Rhabdomyosarcoma is a type of sarcoma. Sarcoma is cancer of soft tissue (such as muscle), connective tissue (such as tendon or cartilage), or bone. Rhabdomyosarcoma usually begins in muscles that are attached to bones and that help the body move, but it may begin in many places in the body. Rhabdomyosarcoma is the most common type of soft tissue sarcoma in children.
There are four main types of rhabdomyosarcoma:
- Embryonal: This type occurs most often in the head and neck area or in the genital or urinary organs, but can occur anywhere in the body. It is the most common type of rhabdomyosarcoma.
- Alveolar: This type occurs most often in the arms or legs, chest, abdomen, genital organs, or anal area.
- Spindle cell/sclerosing: The spindle cell type occurs most often in the paratesticular (testis or spermatic cord) area. There are two other spindle cell/sclerosing subtypes. One is more common in infants and is found in the trunk area. The other can affect children, adolescents, and adults. It is often found in the head and neck area, and is more aggressive.
- Pleomorphic: This is the least common type of rhabdomyosarcoma in children.
See the following PDQ treatment summaries for information about other types of soft tissue sarcoma:
Certain genetic conditions increase the risk of childhood rhabdomyosarcoma.
Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk.
Risk factors for rhabdomyosarcoma include having the following inherited diseases:
- Síndrome de Li-Fraumeni.
- Dicer1 syndrome.
- Neurofibromatosis tipo 1 (NF1).
- Costello syndrome.
- Síndrome de Beckwith-Wiedemann.
- Noonan syndrome.
Children who had a high birth weight or were larger than expected at birth may have an increased risk of embryonal rhabdomyosarcoma.
In most cases, the cause of rhabdomyosarcoma is not known.
A sign of childhood rhabdomyosarcoma is a lump or swelling that keeps getting bigger.
Signs and symptoms may be caused by childhood rhabdomyosarcoma or by other conditions. The signs and symptoms that occur depend on where the cancer forms. Check with your child's doctor if your child has any of the following:
Diagnostic tests and a biopsy are used to diagnose childhood rhabdomyosarcoma.
The diagnostic tests that are done depend in part on where the cancer forms. The following tests and procedures may be used:
- Examen físico e historial médico.: un examen del cuerpo para verificar signos generales de salud, incluida la detección de signos de enfermedad, como bultos o cualquier otra cosa que parezca inusual. También se tomará un historial de los hábitos de salud del paciente y de enfermedades y tratamientos pasados.
- X-ray: An x-ray of the organs and bones inside the body, such as the chest. 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.
- Tomografía computarizada (tomografía computarizada): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, abdomen, pelviso ganglios linfáticos, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A teñir tal vez inyectado en una vena o tragado para ayudar a los órganos o tejidos mostrarse más claramente. Este procedimiento también se denomina tomografía computarizada, tomografía computarizada o tomografía axial computarizada.
Tomografía computarizada (TC). El niño se recuesta sobre una camilla que se desliza a través del escáner, el cual toma una serie de radiografías detalladas de áreas internas del cuerpo.
- Resonancia magnética ( MRI ): Un procedimiento que utiliza un imán, ondas de radio, and a computer to make a series of detailed pictures of areas of the body, such as the cráneo, cerebro, and lymph nodes. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Resonancia magnética (RM). El niño se recuesta en una camilla que se desliza dentro del equipo de resonancia magnética, que toma una serie de imágenes detalladas del interior del cuerpo. La posición del niño en la camilla depende de la parte del cuerpo que se va a examinar.
- Exploración PET (tomografía por emisión de positrones):Un procedimiento para encontrar maligno tumor células en el cuerpo. Una pequena cantidad de radioactive glucose (azúcar) se inyecta en una vena. La mascota escáner rotates around the body and makes a picture of where glucosa is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
Tomografía por emisión de positrones (TEP). El niño se recuesta sobre una camilla que se desliza a través del escáner TEP. El reposacabezas y la correa blanca ayudan al niño a permanecer inmóvil. Se inyecta una pequeña cantidad de glucosa radiactiva (azúcar) en la vena del niño y un escáner genera una imagen de las zonas del cuerpo donde se utiliza la glucosa. Las células cancerosas se ven más brillantes en la imagen porque absorben más glucosa que las células normales.
- Gammagrafía ósea: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of material radioactivo material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner.
Bone scan. A small amount of radioactive material is injected into the child's vein and travels through the blood. The radioactive material collects in the bones. As the child lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen.
- Aspiración y biopsia de médula ósea: La eliminación de médula ósea, blood, and a small piece of bone by inserting a hollow needle into the hipbone. Samples are removed from both hipbones. A patólogo observará la médula ósea, la sangre y el hueso bajo un microscopio to look for signs of cancer.
Aspiración y biopsia de médula ósea. Después de adormecer una pequeña área de piel, se inserta una aguja de médula ósea en el hueso de la cadera del niño. Se extraen muestras de sangre, hueso y médula ósea para examinarlas al microscopio.
- Lumbar puncture: A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs of cancer cells. This procedure is also called an LP or spinal tap.
If these tests show there may be a rhabdomyosarcoma, a biopsy is done. A biopsy is the removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. Because treatment depends on the type of rhabdomyosarcoma, biopsy samples should be checked by a pathologist who has experience in diagnosing rhabdomyosarcoma.
One of the following types of biopsies may be used:
- Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid using a thin needle.
- Core needle biopsy: The removal of tissue using a wide needle. This procedure may be guided using ultrasound, CT scan, or MRI.
- Open biopsy: The removal of tissue through an incision (cut) made in the skin.
- Sentinel lymph node biopsy: The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node in a group of lymph nodes to receive lymphatic drainage from the primary tumor. It is the first lymph node the cancer is likely to spread to from the primary tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. Sometimes, a sentinel lymph node is found in more than one group of nodes. Sentinel lymph node biopsy may be used for patients with rhabdomyosarcoma of the limbs or trunk when enlarged lymph nodes are not found with imaging or physical exam.
The following tests may be done on the sample of tissue that is removed:
- Light microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
- Immunohistochemistry: A laboratory test that uses antibodies to check for certain antigens (markers) in a sample of a patient’s tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to help diagnose cancer and to help tell one type of cancer from another type of cancer.
- FISH (fluorescence in situ hybridization): A laboratory test used to look at and count genes or chromosomes in cells and tissues. Pieces of DNA that contain fluorescent dyes are made in the laboratory and added to a sample of a patient’s cells or tissues. When these dyed pieces of DNA attach to certain genes or areas of chromosomes in the sample, they light up when viewed under a fluorescent microscope. The FISH test is used to help diagnose cancer and help plan treatment.
- Reverse transcription–polymerase chain reaction (RT–PCR) test: A laboratory test in which the amount of a genetic substance called mRNA made by a specific gene is measured. An enzyme called reverse transcriptase is used to convert a specific piece of RNA into a matching piece of DNA, which can be amplified (made in large numbers) by another enzyme called DNA polymerase. The amplified DNA copies help tell whether a specific mRNA is being made by a gene. RT–PCR can be used to check the activation of certain genes that may indicate the presence of cancer cells. This test may be used to look for certain changes in a gene or chromosome, which may help diagnose cancer.
- Cytogenetic analysis: A laboratory test in which the chromosomes of cells in a sample of tissue are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in certain chromosomes may be a sign of cancer. Cytogenetic analysis is used to help diagnose cancer, plan treatment, or find out how well treatment is working.
Hay algunos factores que afectan al pronóstico (es decir, la posibilidad de recuperación) y a las opciones de tratamiento.
El pronóstico y las opciones de tratamiento dependen de lo siguiente:
- The patient's age.
- Where in the body the tumor started.
- The size of the tumor at the time of diagnosis.
- Whether the tumor has been completely removed by surgery.
- The type of rhabdomyosarcoma (embryonal, alveolar, spindle cell/sclerosing, or pleomorphic).
- Whether there are certain changes in the genes.
- Whether the tumor had spread to other parts of the body at the time of diagnosis.
- Whether the tumor was in the lymph nodes at the time of diagnosis.
- Whether the tumor responds to chemotherapy and/or radiation therapy.
For patients with recurrent cancer, prognosis and treatment also depend on the following:
- Where in the body the tumor recurred (came back).
- How much time passed between the end of cancer treatment and when the cancer recurred.
- Whether the cancer was previously treated with radiation therapy.
Stages of Childhood Rhabdomyosarcoma
Puntos clave
- After childhood rhabdomyosarcoma has been diagnosed, treatment is based in part on the stage of the cancer and sometimes it is based on whether all the cancer was removed by surgery.
- Hay tres formas en que el cáncer puede propagarse por el cuerpo.
- El cáncer puede diseminarse desde donde comenzó a otras partes del cuerpo.
- Staging of childhood rhabdomyosarcoma is done in three parts.
- The staging system is based on the size of the tumor, where it is in the body, and whether it has spread to other parts of the body:
- Stage 1
- Stage 2
- Stage 3
- Stage 4
- The grouping system is based on whether the cancer has spread and whether all the cancer was removed by surgery:
- Group I
- Group II
- Group III
- Group IV
- The risk group is based on the staging system and the grouping system.
- Low-risk childhood rhabdomyosarcoma
- Intermediate-risk childhood rhabdomyosarcoma
- High-risk childhood rhabdomyosarcoma
- Sometimes childhood rhabdomyosarcoma continues to grow or comes back after treatment.
After childhood rhabdomyosarcoma has been diagnosed, treatment is based in part on the stage of the cancer and sometimes it is based on whether all the cancer was removed by surgery.
The process used to find out if cancer has spread within the tissue 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 to help find out the stage of the disease.
Treatment for childhood rhabdomyosarcoma is based in part on the stage and sometimes on the amount of cancer that remains after surgery to remove the tumor. The pathologist will use a microscope to check the tissues removed during surgery, including tissue samples from the edges of the areas where the cancer was removed and the lymph nodes. This is done to see if all the cancer cells were taken out during the surgery.
Hay tres formas en que el cáncer puede propagarse por el cuerpo.
El cáncer se puede propagar a través de los tejidos, el sistema linfático y la sangre:
- Tejidos: el cáncer se propaga desde el lugar donde comenzó y crece hacia las áreas cercanas.
- Sistema linfático: el cáncer se propaga desde el lugar donde comenzó hacia el sistema linfático. El cáncer viaja a través de los vasos linfáticos a otras partes del cuerpo.
- Sangre: el cáncer se propaga desde el lugar donde comenzó hacia la la sangre. El cáncer viaja a través de los vasos sanguíneos a otras partes del cuerpo.
El cáncer puede diseminarse desde donde comenzó a otras partes del cuerpo.
Cuando el cáncer se propaga a otra parte del cuerpo, se llama metástasis . Las células cancerosas se desprenden de donde comenzaron (el tumor primario ) y viajan a través del sistema linfático o la sangre.
- Sistema linfático. El cáncer ingresa al sistema linfático, viaja a través de los vasos linfáticos y forma un tumor (tumor metastásico ) en otra parte del cuerpo.
- Sangre. El cáncer ingresa a la sangre, viaja a través de los vasos sanguíneos y forma un tumor (tumor metastásico) en otra parte del cuerpo.
The metastatic tumor is the same type of cancer as the primary tumor. For example, if rhabdomyosarcoma spreads to the lung, the cancer cells in the lung are actually rhabdomyosarcoma cells. The disease is metastatic rhabdomyosarcoma, not lung cancer.
Staging of childhood rhabdomyosarcoma is done in three parts.
Childhood rhabdomyosarcoma is staged by using three different ways to describe the cancer:
- A staging system.
- A grouping system.
- A risk group.
The staging system is based on the size of the tumor, where it is in the body, and whether it has spread to other parts of the body:
Stage 1
In stage 1, the tumor is any size, may have 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 near the ear, nose, sinuses, base of the skull, brain, or spinal cord).
- Gallbladder and bile ducts.
- Ureters or urethra.
- Testes, ovary, vagina, or uterus.
Rhabdomyosarcoma that forms 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 said to be an "unfavorable" site.

Tumor sizes are often measured in centimeters (cm) or inches. Common food items that can be used to show tumor size in cm include: a pea (1 cm), a peanut (2 cm), a grape (3 cm), a walnut (4 cm), a lime (5 cm or 2 inches), an egg (6 cm), a peach (7 cm), and a grapefruit (10 cm or 4 inches).
Stage 2
In stage 2, cancer is found in an "unfavorable" site (any one area not described as "favorable" in stage 1). The tumor is no larger than 5 centimeters and has not spread to lymph nodes.
Stage 3
In stage 3, cancer is found in an "unfavorable" site (any one area not described as "favorable" in stage 1) and one of the following is true:
- The tumor is no larger than 5 centimeters 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 grouping system is based on whether the cancer has spread and whether all the cancer was removed by surgery:
Group I
Cancer was found only in the place where it started and it was completely removed by surgery. Tissue was taken from the edges of where the tumor was removed. This tissue was checked under a microscope by a pathologist and no cancer cells were found.
Group II
Group II is divided into groups IIA, IIB, and IIC.
- IIA: Cancer was removed by surgery but cancer cells were seen when the tissue, taken from the edges of where the tumor was removed, was viewed under a microscope by a pathologist.
- 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, the cancer and lymph nodes were removed by surgery, and at least one of the following is true:
- Tissue taken from the edges of where the tumor was removed was checked under a microscope by a pathologist and cancer cells were seen.
- The furthest lymph node from the tumor that was removed was checked under a microscope by a pathologist and cancer cells were seen.
Group III
Cancer was partly removed by biopsy or surgery but there is tumor remaining that can be seen with the eye.
Group IV
Cancer had spread to distant parts of the body when the cancer was diagnosed.
- Cancer cells are found by an imaging test; or
- There are cancer cells in the fluid around the brain, spinal cord, or lungs, or in fluid in the abdomen; or tumors are found in those areas.
The risk group is based on the staging system and the grouping system.
The risk group describes the chance that rhabdomyosarcoma will recur (come back). Every child treated for rhabdomyosarcoma should receive chemotherapy to decrease the chance cancer will recur. The type of anticancer drug, dose, and the number of treatments given depends on whether the child has low-risk, intermediate-risk, or high-risk rhabdomyosarcoma.
The following risk groups are used:
Low-risk childhood rhabdomyosarcoma
Low-risk childhood rhabdomyosarcoma is one of the following:
- Un embryonal tumor of any size that is found in a "favorable" site. There may be tumor remaining after cirugía that can be seen with or without a microscopio. The cáncer may have spread to nearby ganglios linfáticos. The following areas are "favorable" sites:
- Eye or area around the eye.
- Head or neck (but not in the tissue near the ear, nose, sinuses, base of the skull , brain, or spinal cord).
- Gallbladder and bile ducts.
- Ureter or urethra.
- Testes, ovary, vagina, or uterus.
- An embryonal tumor of any size that is not found in a "favorable" site. There may be tumor remaining after surgery that can be seen only with a microscope. The cancer may have spread to nearby 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 or the alveolar type. It may have spread to nearby lymph nodes and has spread to one or more of the following:
- Other parts of the body that are not near where the tumor first formed.
- Fluid around the brain or spinal cord.
- Fluid in the lung or abdomen.
Sometimes childhood rhabdomyosarcoma continues to grow or comes back after treatment.
Progressive rhabdomyosarcoma is cancer that continues to grow, spread, or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.
Recurrent childhood rhabdomyosarcoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the same place or in other parts of the body, such as the lung, bone, or bone marrow. Less often, rhabdomyosarcoma may come back in the breast in adolescent females or in the liver.
Descripción general de las opciones de tratamiento
Puntos clave
- There are different types of treatment for patients with childhood rhabdomyosarcoma.
- Children with rhabdomyosarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
- Three types of standard treatment are used:
- Cirugía
- Radioterapia
- Quimioterapia
- Se están probando nuevos tipos de tratamiento en ensayos clínicos.
- Inmunoterapia
- Terapia dirigida
- Treatment for childhood rhabdomyosarcoma may cause side effects.
- Los pacientes podrían considerar la posibilidad de participar en un ensayo clínico.
- Los pacientes pueden participar en ensayos clínicos antes, durante o después de comenzar su tratamiento para el cáncer.
- Es posible que se necesiten pruebas de seguimiento.
There are different types of treatment for patients with childhood rhabdomyosarcoma.
Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Debido a que el cáncer en los niños es raro, se debe considerar la posibilidad de participar en un ensayo clínico. Algunos ensayos clínicos están abiertos solo a pacientes que no han iniciado el tratamiento.
Children with rhabdomyosarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
Because rhabdomyosarcoma can form in many different parts of the body, many different kinds of treatments are used. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with rhabdomyosarcoma and who specialize in certain areas of medicine. These may include the following specialists:
Three types of standard treatment are used:
Cirugía
Surgery (removing the cancer in an operation) is used to treat childhood rhabdomyosarcoma. A type of surgery called wide local excision is often done. A wide local excision is the removal of tumor and some of the tissue around it, including the lymph nodes. A second surgery may be needed to remove all the cancer. Whether surgery is done and the type of surgery done depends on the following:
- Where in the body the tumor started.
- The effect the surgery will have on the way the child will look.
- The effect the surgery will have on the child's important body functions.
- How the tumor responded to chemotherapy or radiation therapy that may have been given first.
In most children with rhabdomyosarcoma, it is not possible to remove all of the tumor by surgery.
Rhabdomyosarcoma can form in many different places in the body and the surgery will be different for each site. Surgery to treat rhabdomyosarcoma of the eye or genital areas is usually a biopsy. Chemotherapy, and sometimes radiation therapy, may be given before surgery to shrink large tumors.
After the doctor removes all the cancer that can be seen at the time of the surgery, patients will be given chemotherapy after surgery to kill any cancer cells that are left. Radiation therapy may also be given. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.
Radioterapia
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or stop them from growing. There are two types of radiation therapy:
- Radioterapia externa uses a machine outside the body to send radiation toward the area of the body with cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of external radiation therapy include the following:
- Conformal radiation therapy: Conformal radiation therapy is a type of external radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. This allows a high dose of radiation to reach the tumor and causes less damage to nearby healthy tissue.
- Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles.
- Volumetrical modulated arc therapy (VMAT): VMAT is type of 3-D radiation therapy that uses a computer to make pictures of the size and shape of the tumor. The radiation machine moves in a circle around the patient once during treatment and sends thin beams of radiation of different intensities (strengths) at the tumor. Treatment with VMAT is delivered faster than treatment with IMRT.
- Stereotactic body radiation therapy: Stereotactic body radiation therapy is a type of external radiation therapy. Special equipment is used to place the patient in the same position for each radiation treatment. Once a day for several days, a radiation machine aims a larger than usual dose of radiation directly at the tumor. By having the patient in the same position for each treatment, there is less damage to nearby healthy tissue. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy.
- Proton beam radiation therapy: Proton-beam therapy is a type of high-energy, external radiation therapy. A radiation therapy machine aims streams of protons (tiny, invisible, positively-charged particles) at the cancer cells to kill them. This type of treatment may cause less damage to nearby healthy tissue.
- Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. It is used to treat cancer in areas such as the vagina, vulva, uterus, bladder, prostate, head, or neck. Internal radiation therapy is also called brachytherapy, internal radiation, implant radiation, or interstitial radiation therapy. This approach involves special technical skills and is offered in only a few medical centers.
The type and amount of radiation therapy and when it is given depends on the age of the child, the type of rhabdomyosarcoma, where in the body the tumor started, how much tumor remained after surgery, and whether there is tumor in the nearby lymph nodes.
External radiation therapy is usually used to treat childhood rhabdomyosarcoma but in certain cases internal radiation therapy is used.
Quimioterapia
La quimioterapia es un tratamiento contra el cáncer que utiliza medicamentos para detener el crecimiento de las células cancerosas, ya sea destruyéndolas o impidiendo su división. Cuando la quimioterapia se administra por vía oral o se inyecta en una vena o músculo, los medicamentos ingresan al torrente sanguíneo y pueden alcanzar las células cancerosas de todo el cuerpo ( quimioterapia sistémica ).
Chemotherapy may also be given to shrink the tumor before surgery in order to save as much healthy tissue as possible. This is called neoadjuvant chemotherapy.
Every child treated for rhabdomyosarcoma should receive systemic chemotherapy to decrease the chance the cancer will recur. The type of anticancer drug, dose, and the number of treatments given depends on the age of the child and whether the child has low-risk, intermediate-risk, or high-risk rhabdomyosarcoma.
See Drugs Approved for Rhabdomyosarcoma for more information.
Se están probando nuevos tipos de tratamiento en ensayos clínicos.
Esta sección de resumen describe los tratamientos que se están estudiando en ensayos clínicos. Es posible que no mencione todos los tratamientos nuevos que se están estudiando. La información sobre los ensayos clínicos está disponible en el sitio web del NCI .
Inmunoterapia
Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. There are different types of immunotherapy:
- Vaccine therapy is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat metastatic rhabdomyosarcoma.
- Inhibidor del punto de control inmunitario therapy uses the body's immune system to kill cancer cells. Two types of immune checkpoint inhibitors are being studied in the treatment of childhood rhabdomyosarcoma that has come back after treatment:
- CTLA-4 is a protein on the surface of células T que ayuda a mantener el cuerpo Respuestas inmunes in check. When CTLA-4 attaches to another proteína called B7 on a cancer cell, it stops the T cell from killing the cancer cell. CTLA-4 inhibitors attach to CTLA-4 and allow the T cells to kill cancer cells. Ipilimumab is being studied in the treatment of childhood rhabdomyosarcoma that has come back or progressed during treatment.
Immune checkpoint inhibitor. Checkpoint proteins, such as B7-1/B7-2 on antigen-presenting cells (APC) and CTLA-4 on T cells, help keep the body’s immune responses in check. When the T-cell receptor (TCR) binds to antigen and major histocompatibility complex (MHC) proteins on the APC and CD28 binds to B7-1/B7-2 on the APC, the T cell can be activated. However, the binding of B7-1/B7-2 to CTLA-4 keeps the T cells in the inactive state so they are not able to kill tumor cells in the body (left panel). Blocking the binding of B7-1/B7-2 to CTLA-4 with an immune checkpoint inhibitor (anti-CTLA-4 antibody) allows the T cells to be active and to kill tumor cells (right panel).
- PD-1 and PD-L1 inhibitor therapy: PD-1 is a protein on the surface of T cells that helps keep the body’s immune responses in check. PD-L1 is a protein found on some types of cancer cells. When PD-1 attaches to PD-L1, it stops the T cell from killing the cancer cell. PD-1 and PD-L1 inhibitors keep PD-1 and PD-L1 proteins from attaching to each other. This allows the T cells to kill cancer cells. Nivolumab and pembrolizumab are types of PD-1 inhibitors that are being studied in the treatment of childhood rhabdomyosarcoma that has come back or progressed during treatment.
- CTLA-4 is a protein on the surface of células T que ayuda a mantener el cuerpo Respuestas inmunes in check. When CTLA-4 attaches to another proteína called B7 on a cancer cell, it stops the T cell from killing the cancer cell. CTLA-4 inhibitors attach to CTLA-4 and allow the T cells to kill cancer cells. Ipilimumab is being studied in the treatment of childhood rhabdomyosarcoma that has come back or progressed during treatment.

Inhibidor del punto de control inmunitario. Las proteínas de punto de control, como PD-L1 en las células tumorales y PD-1 en las células T, ayudan a controlar las respuestas inmunitarias. La unión de PD-L1 a PD-1 evita que las células T destruyan células tumorales en el cuerpo (panel izquierdo). El bloqueo de la unión de PD-L1 a PD-1 con un inhibidor del punto de control inmunitario (anti-PD-L1 o anti-PD-1) permite que las células T eliminen las células tumorales (panel derecho).
Terapia dirigida
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. There are different types of targeted therapy:
- mTOR inhibitors stop the protein that helps cells divide and survive. Sirolimus is a type of mTOR inhibitor therapy being studied in the treatment of recurrent rhabdomyosarcoma.
- Tyrosine kinase inhibitors block signals that cancer cells need to grow and divide. MK-1775, cabozantinib-s-malate, and palbociclib are tyrosine kinase inhibitors being studied in the treatment of newly diagnosed or recurrent rhabdomyosarcoma.
Treatment for childhood rhabdomyosarcoma may cause side effects.
Para obtener más información sobre los efectos secundarios que aparecen durante el tratamiento del cáncer, consulte la sección Efectos secundarios.
Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment for rhabdomyosarcoma may include:
- Physical problems that affect the following:
- Teeth, eye, or gastrointestinal function.
- Fertility (ability to have children).
- Cambios en el estado de ánimo, los sentimientos, el pensamiento, el aprendizaje o la memoria.
- Segundos cánceres (nuevos tipos de cáncer).
Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child and the types of symptoms to expect after cancer treatment has ended. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.)
Los pacientes podrían considerar la posibilidad de participar en un ensayo clínico.
Para algunos pacientes, participar en un ensayo clínico puede ser la mejor opción de tratamiento. Los ensayos clínicos son parte del proceso de investigación del cáncer y se realizan para determinar si los nuevos tratamientos para el cáncer son seguros y eficaces o mejores que el tratamiento estándar.
Muchos de los tratamientos estándar actuales para el cáncer se basan en ensayos clínicos anteriores. Los pacientes que participan en un ensayo clínico pueden recibir el tratamiento estándar o ser de los primeros en recibir uno nuevo.
Los pacientes que participan en ensayos clínicos también ayudan a mejorar la forma en que se tratará el cáncer en el futuro. Incluso cuando los ensayos clínicos no conducen a nuevos tratamientos efectivos, suelen responder preguntas clave y contibuir de forma significativa al avance de la investigación.
Los pacientes pueden participar en ensayos clínicos antes, durante o después de comenzar su tratamiento para el cáncer.
En algunos ensayos clínicos solo pueden participar aquellos pacientes que aún no han recibido tratamiento. Otros ensayos prueban tratamientos para pacientes con cáncer que no han mostrado una mejoría. También hay ensayos que prueban nuevas formas de evitar una recidiva (regreso) del cáncer o de reducir los efectos secundarios del tratamiento del cáncer.
Se están realizando ensayos clínicos en muchas partes del país. Puede encontrar información sobre los ensayos clínicos patrocinados por el National Cancer Institute en la página web de búsqueda de ensayos clínicos del NCI. Puede encontrar ensayos clínicos patrocinados por otras organizaciones en el sitio web ClinicalTrials.gov.
Es posible que se necesiten pruebas de seguimiento.
A medida que su hijo se somete al tratamiento, se le harán pruebas o controles de seguimiento. Es posible que se repitan algunas pruebas que se realizaron para diagnosticar o estadificar el cáncer para ver qué tan bien está funcionando el tratamiento. Las decisiones sobre si continuar, cambiar o suspender el tratamiento pueden basarse en los resultados de estas pruebas.
Algunas de las pruebas se seguirán realizando periódicamente después de que finalice el tratamiento. Los resultados de estas pruebas pueden mostrar si la afección de su hijo ha cambiado o si el cáncer ha reaparecido (recaído).
Treatment of Childhood Rhabdomyosarcoma
The treatment of newly diagnosed childhood rhabdomyosarcoma often includes surgery, radiation therapy, and chemotherapy. The order that these treatments are given depends on where in the body the tumor started, the size of the tumor, the type of tumor, and whether the tumor has spread to lymph nodes or other parts of the body. See the Treatment Option Overview section of this summary for more information about surgery, radiation therapy, and chemotherapy used to treat children with rhabdomyosarcoma.
Rhabdomyosarcoma of the brain and head and neck
- For tumors of the brain: Treatment may include surgery to remove the tumor, radiation therapy, and chemotherapy.
- For tumors of the head and neck that are in or near the eye: Treatment may include 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 are near the ear, nose, sinuses, or base of the skull but not in or near the eye: Treatment may include radiation therapy and chemotherapy.
- For tumors of the head and neck that are not in or near the eye and not near the ear, nose, sinuses, or base of the skull: Treatment may include chemotherapy, radiation therapy, and surgery to remove the tumor.
- For tumors of the head and neck that cannot be removed by surgery: Treatment may include chemotherapy and radiation therapy including stereotactic body radiation therapy.
- For tumors of the larynx (voice box): Treatment may include chemotherapy and radiation therapy. Surgery to remove the larynx is usually not done, so that the voice is not harmed.
Rhabdomyosarcoma of the arms or legs
- Chemotherapy followed by surgery to remove the tumor. If the tumor was not completely removed, a second surgery to remove the tumor may be done. Radiation therapy may also be given.
- For tumors of the hand or foot, radiation therapy and chemotherapy may be given. The tumor may not be removed because it would affect the function of the hand or foot.
- Lymph node dissection (one or more ganglios linfáticos are removed and a sample of tejido is checked under a microscopio para señales de cáncer).
- For tumors in the arms, lymph nodes near the tumor and in the armpit area are removed.
- For tumors in the legs, lymph nodes near the tumor and in the groin area are removed.
Rhabdomyosarcoma of the chest, abdomen, or pelvis
- For tumors in the chest or abdomen (including the chest wall or abdominal wall): Surgery (wide local excision) may be done. If the tumor is large, chemotherapy and radiation therapy are given to shrink the tumor before surgery.
- For tumors of the pelvis: Surgery (wide local excision) may be done. If the tumor is large, chemotherapy is given to shrink the tumor before surgery. Radiation therapy may be given after surgery.
- For tumors of the diaphragm: A biopsy of the tumor is followed by chemotherapy and radiation therapy to shrink the tumor. Surgery may be done later to remove any remaining cancer cells.
- For tumors of the gallbladder or bile ducts: A biopsy of the tumor is followed by chemotherapy and radiation therapy. Surgery may be done later to remove any remaining cancer cells.
- For tumors of the muscles or tissues around the anus or between the vulva and the anus or the scrotum and the anus: Surgery may be done to remove as much of the tumor as possible and some nearby lymph nodes, followed by chemotherapy and radiation therapy.
Rhabdomyosarcoma of the kidney
- For tumors of the kidney: Surgery to remove as much of the tumor as possible. Chemotherapy and radiation therapy may also be given.
Rhabdomyosarcoma of the bladder or prostate
- For tumors that are only at the top of the bladder: Surgery (wide local excision) is done.
- For tumors of the próstata or bladder (other than the top of the bladder):
- Chemotherapy and radiation therapy are given first to shrink the tumor. If cancer cells remain after chemotherapy and radiation therapy, the tumor is removed by surgery. 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).
- Chemotherapy is given first to shrink the tumor. Surgery to remove the tumor, but not the bladder or prostate, is done. Internal or external radiation therapy may be given after surgery.
- Surgery to remove the tumor, but not the bladder or prostate. Internal radiation therapy is given after surgery.
Rhabdomyosarcoma of the area near the testicles
- Surgery to remove the testicle and spermatic cord. The lymph nodes in the back of the abdomen may be checked for cancer, especially if the lymph nodes are large. Patients older than 10 years with no sign of enlarged lymph nodes in the back of the abdomen should have a nerve-sparing retroperitoneal lymph node dissection.
- Radiation therapy may be given if the tumor cannot be completely removed by surgery.
Rhabdomyosarcoma of the vulva, vagina, uterus, or ovary
- For tumors of the vulva and vagina: Treatment may include chemotherapy followed by surgery to remove the tumor. Internal or external radiation therapy may be given after surgery.
- For tumors of the uterus: Treatment may include chemotherapy with or without radiation therapy. Sometimes surgery may be needed to remove any remaining cancer cells.
- For tumors of the ovary: Treatment may include chemotherapy followed by surgery to remove any remaining tumor.
Clinical Trials For Childhood Rhabdomyosarcoma
- A clinical trial of combination chemotherapy with or without temsirolimus.
- A clinical trial of targeted therapy with cabozantinib-s-malate.
Metastatic rhabdomyosarcoma
Treatment, such as chemotherapy followed by radiation therapy or surgery to remove the tumor, is given to the site where the tumor first formed. If the cancer has spread to the brain, spinal cord, or lungs, radiation therapy may also be given to the sites where the cancer has spread.
The following treatment is being studied for metastatic rhabdomyosarcoma:
Puede utilizar la búsqueda de ensayos clínicos y encontrar ensayos clínicos sobre cáncer patrocinados por el NCI que acepten participantes. La búsqueda le permite filtrar los ensayos según el tipo de cáncer, su edad y el lugar donde se realizan los ensayos. También encontrará información general sobre los ensayos clínicos.
Treatment of Progressive or Recurrent Childhood Rhabdomyosarcoma
Para obtener información sobre los tratamientos que se enumeran a continuación, consulte la sección Descripción general de las opciones de tratamiento.
Treatment options for progressive or recurrent childhood rhabdomyosarcoma are based on many factors, including where in the body the cancer has come back, what type of treatment the child had before, and the needs of the child.
Treatment of progressive or recurrent rhabdomyosarcoma may include one or more of the following:
- Cirugía.
- Radioterapia.
- Quimioterapia.
- A clinical trial of combination chemotherapy with or without temsirolimus.
- A clinical trial of targeted therapy or immunotherapy (sirolimus, ipilimumab, nivolumab, or pembrolizumab).
- A clinical trial of targeted therapy with a tyrosine kinase inhibitor (MK-1775, cabozantinib-s-malate, or palbociclib) and chemotherapy.
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
- New therapies being studied in early stage clinical trials should be considered for patients with recurrent rhabdomyosarcoma.
Puede utilizar la búsqueda de ensayos clínicos y encontrar ensayos clínicos sobre cáncer patrocinados por el NCI que acepten participantes. La búsqueda le permite filtrar los ensayos según el tipo de cáncer, su edad y el lugar donde se realizan los ensayos. También encontrará información general sobre los ensayos clínicos.
To Learn More About Childhood Rhabdomyosarcoma
For more information from the National Cancer Institute about childhood rhabdomyosarcoma, see the following:
- Página de inicio sobre el sarcoma de tejidos blandos
- Tomografías computarizadas (TC) y cáncer
- Drugs Approved for Rhabdomyosarcoma
- Inmunoterapia para tratar el cáncer
- Terapias dirigidas contra el cáncer
Para obtener más información sobre el cáncer infantil y otros recursos generales sobre el cáncer, consulte:
- Sobre el cáncer
- Cánceres infantiles
- CureSearch para el cáncer infantil
- Efectos tardíos del tratamiento del cáncer infantil
- Adolescentes y adultos jóvenes con cáncer
- Niños con cáncer: una guía para padres
- El cáncer en los niños y los adolescentes
- Estadificación del cáncer
- Cómo hacer frente al cáncer
- Preguntas para hacerle a su médico sobre el cáncer
- Para supervivientes, cuidadores e intercesores
Información sobre este resumen del PDQ
Acerca del PDQ
Physician Data Query (PDQ) es la base de datos integral sobre el cáncer del Instituto Nacional del Cáncer (NCI). La base de datos del PDQ contiene resúmenes de la última información publicada sobre prevención, detección, genética, tratamiento, atención médica de apoyo y medicina complementaria y alternativa relacionada con el cáncer. La mayoría de los resúmenes se redactan en dos versiones. Las versiones para profesionales de la salud contienen información detallada escrita en lenguaje técnico. Las versiones para pacientes están redactadas en un lenguaje fácil de entender, que no es técnico. Ambas versiones contienen información sobre el cáncer correcta y actualizada. La mayoría de las versiones también están disponibles en español.
El PDQ es un servicio del NCI. El NCI es parte de los Institutos Nacionales de Salud (NIH), que son el centro de investigación biomédica del Gobierno federal. Los resúmenes del PDQ se basan en una revisión independiente de la literatura médica. No son declaraciones de políticas del NCI ni de los NIH.
Propósito de este resumen
This PDQ cancer information summary has current information about the treatment of childhood rhabdomyosarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Revisores y actualizaciones
Los consejos editoriales escriben los resúmenes de información sobre el cáncer del PDQ y los mantienen actualizados. Estos consejos están conformados por equipos de especialistas en el tratamiento del cáncer y otras especialidades relacionadas con esta enfermedad. Los resúmenes se revisan periódicamente y se modifican cuando hay información nueva. La fecha de actualización al pie de cada resumen indica cuándo fue el cambio más reciente.
La información de este resumen para pacientes procede de la versión para profesionales de la salud, la cual el Consejo editorial del PDQ sobre el tratamiento pediátrico revisa y actualiza de forma periódica según sea necesario.
Información sobre ensayos clínicos
Un ensayo clínico es un estudio para responder a una pregunta científica; por ejemplo, si un tratamiento es mejor que otro. Los ensayos se basan en estudios anteriores y en lo que se ha aprendido en el laboratorio. Cada ensayo responde a ciertas preguntas científicas para descubrir mejores maneras de ayudar a los pacientes con cáncer. Durante los ensayos clínicos de tratamiento, se recopila información sobre los efectos de un tratamiento nuevo y su eficacia. Si un ensayo clínico demuestra que un tratamiento nuevo es mejor que uno que se utiliza actualmente, el tratamiento nuevo puede convertirse en “estándar”. Participar en un ensayo clínico puede ser una opción para los pacientes. Algunos ensayos clínicos están abiertos solo a pacientes que aún no han comenzado el tratamiento.
Los ensayos clínicos se pueden encontrar en línea en el sitio web del NCI. Para obtener más información, llame al Servicio de Información sobre el Cáncer (CIS, por sus siglas en inglés), el centro de contacto del NCI, al 1-800-4-CANCER (1-800-422-6237).
Permiso de uso de este resumen
PDQ (Physician Data Query) es una marca registrada. Se autoriza el libre uso del contenido de los documentos del PDQ como texto. Sin embargo, no se podrá identificar como un resumen de información sobre cáncer del PDQ del NCI, salvo que se reproduzca en su totalidad y se actualice con regularidad. Por otra parte, se permite que los autores incluyan una oración como: “En el resumen del PDQ del NCI sobre la prevención del cáncer de mama se describen, de manera concisa, los siguientes riesgos: [incluir fragmento del resumen]”.
La forma recomendada para citar este resumen del PDQ es:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Rhabdomyosarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389279]
Las imágenes de este resumen se utilizan con el permiso del autor, artista y/o editorial para uso exclusivo en los resúmenes del PDQ. Si desea usar una imagen de un resumen del PDQ sin incluir el resumen completo, debe obtener autorización del propietario. El National Cancer Institute no puede otorgar dicho permiso. Para obtener más información sobre el uso de las imágenes de este resumen o de otras ilustraciones relacionadas con el cáncer, consulte Visuals Online, una colección de más de 3,000 imágenes científicas.
Descargo de responsabilidad
La información de estos resúmenes no debe utilizarse para tomar decisiones sobre reembolsos de seguros. Puede encontrar más información sobre la cobertura de seguros disponible en Cancer.gov/espanol en la página Manejo de la atención médica del cáncer.
Contáctenos
Puede encontrar más información sobre cómo contactarnos o recibir ayuda en el sitio web Cancer.gov en la página Contact Us for Help. También puede enviar sus preguntas a Cancer.gov en el apartado E-mail Us del sitio web.
Updated:
Source URL: https://www.cancer.gov/node/5282/syndication
Agencia de origen: National Cancer Institute (NCI)
Captured Date: 2013-09-14 09:02:45.0