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Rhabdomyosarcoma - Childhood - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 02/2014

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Childhood Rhabdomyosarcoma. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

About sarcoma

Soft tissue sarcoma is a cancer that develops in the tissues that support and connect the body. It can occur in fat, muscle, nerves, tendons, joints, blood vessels, or lymph vessels. Cancer begins when normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can spread to other parts of the body. A benign tumor means the tumor will not spread. In general, sarcoma is uncommon, accounting for about 1% of all cancers.

About rhabdomyosarcoma

Rhabdomyosarcoma is a type of soft tissue sarcoma (STS) that begins in mesenchymal cells, which are immature cells that normally become muscle. This disease develops in a type of muscle called striated muscle. Striated muscles are the skeletal voluntary muscles, which are those muscles that people can control.

Rhabdomyosarcoma can occur anywhere in the body:

  • Head and neck: about 39% of all rhabdomyosarcoma cases. This includes parameningeal sites, near the membranes covering the brain, 24%; eye socket, 8%; and other head and neck locations, 7%.
  • Urinary or reproductive organs: about 29% of cases
  • Arms or legs: about 15% of cases
  • Other sites: about 17% of cases. This includes the trunk (torso), intrathoracic (inside the lung and/or chest), biliary tract, retroperitoneal, pelvic, and perineal sites (close to the anus, vagina, and urinary structures).

Types of rhabdomyosarcoma

Each rhabdomyosarcoma tumor is classified as either favorable or unfavorable based on its histology, which is what the cells look like under a microscope. The terms “favorable” and “unfavorable” refer to the appearance of the cancer cells. In general, the more cancer cells look like normal cells, the more “favorable” they are and the greater the chance that treatment will be successful.

Favorable histology tumors include the following:

  • Embryonal rhabdomyosarcoma.This is the most common type of rhabdomyosarcoma, frequently found in the head and neck and in the reproductive and urinary organs.
    • Botryoid rhabdomyosarcoma. This is one subtype of embryonal rhabdomyosarcoma, found most often in hollow organs, such as the bladder or a girl’s vagina.
    • Spindle rhabdomyosarcoma. This is another subtype of embryonal rhabdomyosarcoma, found most often in the area around a boy’s testicles.

Unfavorable histology tumors include the following:

  • Alveolar rhabdomyosarcoma. This is a more aggressive type of rhabdomyosarcoma, found most often in an arm, leg, or the trunk of the body.
  • Pleomorphic and undifferentiated rhabdomyosarcoma. These are the rarest types of rhabdomyosarcoma, found most commonly in an arm, leg, or the body’s trunk.

Rhabdomyosarcoma is most often found in children and young adults. This section covers rhabdomyosarcoma diagnosed in children. Learn more about soft tissue sarcoma in adults.

Looking for More of an Overview?

If you would like additional introductory information, explore these related items. Please note these links take you to other sections on Cancer.Net:

  • ASCO Answers Fact Sheet: Read a one-page fact sheet (available as a PDF) that offers an easy-to-print introduction to this type of cancer.
  • Cancer.Net Patient Education Videos:  View short videos on childhood cancer and sarcoma led by ASCO experts that provide basic information and areas of research.

To continue reading this guide, use the menu on the side of your screen to select another section.

Rhabdomyosarcoma - Childhood - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 02/2014

ON THIS PAGE: You will find information about how many people learn they have this type of cancer each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

About 350 people are diagnosed with rhabdomyosarcoma each year. More than half of childhood rhabdomyosarcomas are diagnosed in those under age 10. Rhabdomyosarcoma accounts for 3% of all new childhood cancers each year in the United States. It is the most common soft tissue sarcoma in children.

The overall five-year survival rate is the percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases. For children younger than 14, this is 64%, but it varies depending on the tumor location, stage and risk group, and the child’s age.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of people with this type of cancer, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with rhabdomyosarcoma. Because the survival statistics are measured in five-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer. Learn more about understanding statistics.

Source: The American Cancer Society.

To continue reading this guide, use the menu on the side of your screen to select another section.  

Rhabdomyosarcoma - Childhood - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 02/2014

ON THIS PAGE: You will find out more about the factors that increase the chance of developing this type of cancer. To see other pages, use the menu on the side of your screen.

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do.

Doctors and researchers don’t know what causes most childhood cancers. In most cases of rhabdomyosarcoma, there are no clear risk factors. However, it has been shown that children who have the following rare, inherited conditions have a higher risk of developing rhabdomyosarcoma:

  • Costello syndrome
  • Cardio-facio-cutaneous syndrome

A few cases of rhabdomyosarcoma have been associated with some problems that occur in development before a child is born, called congenital anomalies. Parental use of cocaine and marijuana before a child’s birth may increase a child’s risk of developing rhabdomyosarcoma.

To continue reading this guide, use the menu on the side of your screen to select another section.

Rhabdomyosarcoma - Childhood - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 02/2014

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

Children with rhabdomyosarcoma may experience the following symptoms or signs. Sometimes, children with rhabdomyosarcoma do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer.

Because rhabdomyosarcoma occurs most often in areas that cause noticeable symptoms, it is often diagnosed early. A small, visible, painless lump often forms near the surface of the body, where it is more easily spotted. The symptoms of a less obvious tumor can vary depending on where it is located in the body. A larger tumor may cause pain if it is pushing on nerves or other areas of the body.

Location of Tumor

Symptom

Nasal cavity

Nosebleed

Symptoms similar to a sinus infection

Ear

Earaches, bleeding, or discharge from the ear canal

Mass growing from the ear canal

Behind the eye

May cause the eye to bulge or swell

May make the child look cross-eyed

Bladder, urinary tract, vagina, or testicle

May cause blood in the urine and make urinating difficult

Bleeding from the vagina

Mass growing from the vagina

Rapid growth around the testicles

Abdomen or pelvis

Abdominal pain

Vomiting

Constipation

Arm or leg muscle

Mass, growth, lump, bump, or swelling in the leg or arm that may or may not be painful

If the cancer has spread, the child may experience a chronic cough, bone pain, enlarged lymph nodes, weakness, or weight loss.

If you are concerned about one or more of the symptoms or signs on this list, please talk with your child’s doctor. Your doctor will ask how long and how often your child has been experiencing the symptom(s), in addition to other questions. This is to help find out the cause of the problem, called a diagnosis.

If cancer is diagnosed, relieving symptoms remains an important part of cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your child’s health care team about symptoms your child experiences, including any new symptoms or a change in symptoms.

The next section helps explain what tests and scans may be needed to learn more about the cause of the symptoms. Use the menu on the side of your screen to select Diagnosis, or you can select another section, to continue reading this guide.

Rhabdomyosarcoma - Childhood - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 02/2014

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose cancer and find out if it has spread to another part of the body, called metastasis. Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has spread. This list describes options for diagnosing this type of cancer, and not all tests listed will be used for every person. Your child’s doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition
  • Type of cancer suspected
  • Signs and symptoms
  • Previous test results

In addition to a physical examination, the following tests may be used to diagnose rhabdomyosarcoma:

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The type of biopsy performed will depend on the location of the cancer. If the tumor is near the surface of the body, the child will be given a local anesthetic to numb the area during the procedure; if it is deeper inside the body, a general anesthetic (medication to block the awareness of pain) will be used. The sample removed during the biopsy is analyzed by a pathologist. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

Immunocytochemistry tests. These are special stains done on the cells taken during the biopsy to help the doctor make an accurate diagnosis of rhabdomyosarcoma. Stains that show muscle cell development, including actin, desmin, MyoD-1 and Myogenin, are most helpful.

Genetic tests of tumor tissue. Chromosomes are the structures that contain the genes in a cell. Changes in certain chromosomes in the tumor cells, called chromosomal translocations, can help doctors identify the alveolar subtype of rhabdomyosarcoma (see Overview), although some alveolar rhabdomyosarcomas lack any specific translocation. Doctors will do a genetic test of the tumor tissue to see if translocations have occurred.

Bone marrow biopsy. The doctor may also perform a bone marrow biopsy. Rhabdomyosarcoma can spread to the bone marrow, and only a biopsy can detect it there. Bone marrow has both a solid and a liquid part. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle. The sample(s) are then analyzed by a pathologist. A common site for a bone marrow biopsy is the pelvic bone, which is located in the lower back by the hip. The skin in that area is usually numbed with medication beforehand, and other types of anesthesia may be used.

Imaging tests

To determine where the cancer is located and if it has spread, the doctor may use the following imaging tests:

X-ray. An x-ray is a way to create a picture of the structures inside of the body using a small amount of radiation.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A CT scan can also be used to measure the tumor’s size. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a pill to swallow.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. MRI can also be used to measure the tumor’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a pill to swallow.

Bone scan. A bone scan uses a radioactive tracer to look at the inside of the bones. The tracer is injected into a patient’s vein. It collects in areas of the bone and is detected by a special camera. Healthy bone appears gray to the camera, and areas of injury, such as those caused by cancer, appear dark.

Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive sugar substance is injected into the patient’s body. This sugar substance is taken up by cells that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body.

After diagnostic tests are done, your child’s doctor will review all of the results with you. If the diagnosis is cancer, these results also help the doctor describe the cancer; this is called the stage.

The next section helps explain the different stages for this type of cancer. Use the menu on the side of your screen to select Stages, or you can select another section, to continue reading this guide.

Rhabdomyosarcoma - Childhood - Stages and Groups

This section has been reviewed and approved by the Cancer.Net Editorial Board, 02/2014

ON THIS PAGE: You will learn about how doctors describe a cancer’s growth or spread. This is called the stage. The tumor’s Clinical Group describes whether it can be removed by surgery. To see other pages, use the menu on the side of your screen.

Doctors use both a grouping system and a staging system to describe rhabdomyosarcoma. Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out the cancer’s stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient’s prognosis, which is the chance of recovery. There are different stage descriptions for different types of cancer.

Stage is determined by information called the TNM system. This system judges three factors: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to other parts of the body. The results are combined to determine the stage of cancer for each person.

TNM is an abbreviation for tumor (T), node (N), and metastasis (M): the “T” is used to describe the size and location of the tumor; the “N” stands for lymph nodes, the tiny bean-shaped organs that help fight infection; the “M” indicates whether the cancer has spread to other parts of the body.

The Clinical Group is based on whether a surgeon can remove the tumor. The Intergroup Rhabdomyosarcoma Studies (IRS) designed the following grouping and staging guidelines:

IRS Clinical Group

Group I: Describes a tumor that can be completely removed by surgery.

Group II: Describes a tumor that has been removed with surgery, but cancer cells remain at the edge of the tissue that surrounded the tumor, called a margin, and/or cancer cells are in the regional lymph nodes, which are lymph nodes near the site of the tumor.

Group III: Describes a local tumor, which is a tumor that has not spread outside of the area where it started, that cannot be removed by surgery.

Group IV: Distant metastases, which means that cancer that has spread to other parts of the body, are present at the time of diagnosis.

IRS-modified TNM stage

Stage 1: Describes a small local tumor in the orbit (the area near the eye); head and neck area, except for parameningeal sites; or a genitourinary tract tumor, except for a tumor in the bladder or prostate.

Stage 2: Describes a small local tumor in any part of the body not in Stage 1. The tumor is smaller than 5 centimeters (cm), and there is no spread to regional lymph nodes.

Stage 3: Describes a local tumor in any part of the body not included in Stage 1 that is larger than 5 cm in diameter and/or has spread to regional lymph nodes.

Stage 4: Distant metastases are present at diagnosis. The primary tumor can be of any size or location.

Recurrent: Recurrent cancer is cancer that has come back after treatment. If there is a recurrence, the cancer may need to be staged again (called re-staging) using the system above.

Source: Soft Tissue Sarcoma Committee of the Children's Oncology Group (COG).

Information about the cancer’s stage will help the doctor recommend a treatment plan.  The next section helps explain the treatment options for this type of cancer. Use the menu on the side of your screen to select Treatment Options, or you can select another section, to continue reading this guide.

Rhabdomyosarcoma - Childhood - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 02/2014

ON THIS PAGE: You will learn about the different ways doctors use to treat children with this type of cancer. To see other pages, use the menu on the side of your screen.

In general, cancer in children is uncommon, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That’s why more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare the standard treatments (the best proven treatments available) with newer approaches to treatments that may be more effective. Clinical trials may test such approaches as a new drug, a new combination of standard treatments, or new doses of current therapies. Studying new treatments involves careful monitoring using scientific methods and all participants are followed closely to track their health and progress. The Children’s Oncology Group (COG) conducts national clinical trials that are available for children with rhabdomyosarcoma. COG is a National Cancer Institute-supported clinical trials cooperative group devoted exclusively to childhood and adolescent cancer research.

To take advantage of these newer treatments, all children with cancer should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children with cancer and have access to the latest research. A doctor who specializes in treating children with cancer is called a pediatric oncologist. In many cases, a team of doctors works with a child and the family to provide care; this is called a multidisciplinary team. Pediatric cancer centers often have extra support services for children and their families, such as child life specialists, nutritionists, physical and occupational therapists, social workers, and counselors. Special activities and programs to help your child and family cope may also be available.

Descriptions of the most common treatment options for rhabdomyosarcoma are listed below. Children and adolescents with rhabdomyosarcoma require multidisciplinary therapy planning, which means using combinations of chemotherapy, surgery, and/or radiation therapy. All children with rhabdomyosarcoma require chemotherapy, as well as surgery and/or radiation therapy. Treatment options and recommendations depend on several factors, including the subtype, stage, and group of rhabdomyosarcoma, possible side effects, and the family’s preferences and overall health. Your child’s care plan may also include treatment for symptoms and side effects, an important part of cancer care.

Take time to learn about all of your child’s treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with the doctor and what your child can expect while receiving the treatment. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and surrounding tissue during an operation, leaving a negative margin. This means there is no trace of cancer in the healthy tissue around the tumor’s location. A surgical oncologist is a doctor who specializes in treating cancer using surgery. Learn more about the basics of cancer surgery.

Even children who have a rhabdomyosarcoma tumor that can be completely removed by surgery still require chemotherapy (see below).

An inoperable tumor is when the tumor cannot be completely removed or is unable to be removed by surgery. If the tumor is inoperable, the doctor will recommend a different treatment plan with chemotherapy and radiation therapy to destroy the cancer cells. Also, a biopsy is needed to determine the type of tumor (see Diagnosis).

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Chemotherapy is given by a pediatric or medical oncologist, a doctor who specializes in treating cancer with medication. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.

Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Chemotherapy for rhabdomyosarcoma is given by injection into a vein (called intravenous or IV chemotherapy). The drugs that are used most often in North America for rhabdomyosarcoma are vincristine (Oncovin, Vincasar), dactinomycin (Cosmegen, Lyovac Cosmegen), and cyclophosphamide (Cytoxan, Clafen, Neosar). This combination is called VAC.

Different studies are underway to determine the best medications and doses, and often these studies are classified based on the likelihood (or the risk) that the disease will recur (come back). The tumor may be classified as one of these three categories about its recurrence risk:

  • low risk,
  • intermediate risk, or
  • high risk

The COG recently completed a research study for patients with low-risk rhabdomyosarcoma (started in August 2004) evaluating four cycles of VAC followed by vincristine and dactinomycin (called VA), in an attempt to decrease the total amount of medication given. In addition, for patients who had tumor cells remaining after surgery, this study evaluated a lower dose of radiation therapy. Early results for the patients with the lowest risk rhabdomyosarcoma (stage 1 or 2, group I or II with embryonal tumors, or stage 1, group III orbital embryonal tumors) show that the lower amount of medication had the same outcome as prior therapies. The long-term side effects for patients who received chemotherapy and radiation therapy are still being followed.

The COG recently completed and closed the clinical trial for patients with intermediate-risk rhabdomyosarcoma, started in 2006 and closed in 2013. The clinical trial tested whether the combination of irinotecan (Camptosar) and vincristine is more effective when added to VAC. In this study, a lower dose of cyclophosphamide is used in VAC cycles. This study also evaluated the use of radiation therapy early in the treatment process. The results of this clinical trial are not yet available.

A COG study for patients with high-risk rhabdomyosarcoma completed in 2008 evaluated the effectiveness of irinotecan and vincristine as initial treatment, followed by treatment with alternating cycles of a combination of doxorubicin (Adriamycin) and cyclophosphamide, and a combination of ifosfamide (Cyfos, Ifes, Ifosfamidum) and etoposide (VePesid, Toposar), given every two weeks. This therapy was then followed by four cycles of VAC.

The chemotherapy combination from this study is the basis the just-completed pilot study that opened in January 2010 for patients with high-risk disease and is evaluating whether the addition of temozolamide (Temodar) to vinicristine and irinotecan and/or the addition of IMC-A12 (Cixutumumab) is safe and potentially more effective than the base combination therapy.

Learn more about current COG studies for rhabdomyosarcoma. (Please note that this link will take you off Cancer.Net to another website.)

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. The side effects most common for the specific drugs used to treat rhabdomyosarcoma are listed below. Talk with your health care team about what your child may experience based on his or her specific treatment plan, and how side effects will be relieved or managed.

VAC therapy: Nausea, vomiting, and bone marrow suppression, which means the child’s immune system functioning may be reduced, raising the risk of infection and other problems. Although very rare, a few patients may have significant liver damage.

Doxorubicin: Nausea, vomiting, bone marrow suppression, and sores in the mouth. Rarely, this drug affects heart function.

Ifosfamide and etoposide: Nausea, vomiting, bone marrow suppression, and decreased kidney function.

Irinotecan: Loose bowel movements.

Learn more about managing common side effects of cancer treatment. Most side effects usually go away once treatment is finished. However, intensive therapy used for intermediate- and high-risk rhabdomyosarcoma may cause permanent infertility, which is the inability to have a child. Talk with your doctor about this possibility and whether your family should talk with a fertility specialist before cancer treatment begins.

Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation therapy given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time.

Side effects from radiation therapy include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished. Learn more about radiation therapy.

Getting care for symptoms and side effects

Cancer and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care can help a person at any stage of illness. People often receive treatment for the cancer and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, and other therapies. Your child may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, and radiation therapy. Talk with the doctor about the goals of each treatment in your treatment plan.

Before treatment begins, talk with the health care team about the possible side effects of your child’s specific treatment plan and supportive care options. And during and after treatment, be sure to tell the doctor or another health care team member if your child is experiencing a problem so it is addressed as quickly as possible. Learn more about palliative care

Remission and the chance of recurrence

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called “no evidence of disease” or NED. 

A remission can be temporary or permanent. This uncertainty leads to many patients and families feeling worried or anxious that the cancer will come back. While many remissions are permanent, it’s important to talk with your child’s doctor about the possibility of the cancer returning. As outlined above, the disease may be classified according to its risk of returning -- as low-risk, intermediate-risk, or high-risk disease -- and this may have been a factor in the child’s treatment plan. Understanding the risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence

If the cancer does return after the original treatment, it is called recurrent cancer. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

When this occurs, testing will begin again to learn as much as possible about the recurrence, including whether the cancer’s stage and group have changed. After testing is done, your child’s doctor will talk with you about treatment options. The treatment plan may involve chemotherapy, but it depends on how much of the tumor can be surgically removed, where the cancer recurred, and the treatment the child received previously. Often, new experimental treatments for recurrent rhabdomyosarcoma are offered at centers that specialize in this disease.

People with recurrent cancer often experience emotions such as disbelief or fear. Patients and their families are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with cancer recurrence.

If treatment fails

Although treatment is successful for the majority of children with cancer, sometimes it is not. If a child’s cancer cannot be cured or controlled, this is called advanced or terminal cancer. In rare cases, this may occur during the original diagnosis and treatment or after initial therapy has been completed and there is a recurrence. The likelihood of treatment failure tends to be higher in children with higher risk disease at the time of the original diagnosis.

This diagnosis is stressful, and it may be difficult to discuss. However, it is important to have open and honest conversations with your child’s doctor and health care team to express your family’s feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families.

Parents or guardians are encouraged to think about where the child would be most comfortable: at home, in a home-like setting elsewhere, in the hospital, or in a hospice environment. Hospice care is a type of palliative care for people who are expected to live less than six months. It is designed to provide the best possible quality of life for people who are near the end of life. Nursing care and special equipment can make staying at home a workable alternative for many families. Some children may be happier if they can arrange to attend school part-time or keep up other activities and social connections. The child’s health care team can help parents or guardians decide on an appropriate level of activity. Making sure a child is physically comfortable and free from pain is extremely important as part of end-of-life care. Learn more about caring for a terminally ill child and advanced cancer care planning.

The death of a child is an enormous tragedy, and families may need support to help them cope with the loss. Pediatric cancer centers often have professional staff and support groups to help with the process of grieving. Learn more on grieving the loss of a child.

The next section helps explain clinical trials, which are research studies. Use the menu on the side of your screen to select About Clinical Trials, or you can select another section, to continue reading this guide.

Rhabdomyosarcoma - Childhood - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 02/2014

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. To see other pages, use the menu on the side of your screen.

Doctors and scientists are always looking for better ways to treat children with rhabdomyosarcoma. To make scientific advances, doctors create research studies involving volunteers, called clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Children who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and manage the late effects that may occur after treatment. Talk with your child’s doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease.

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating rhabdomyosarcoma. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with rhabdomyosarcoma.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

To join a clinical trial, parents and children must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

For specific topics being studied for rhabdomyosarcoma, learn more in the Latest Research section.

Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that patients participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if the patient chooses to leave the clinical trial before it ends. 

Cancer.Net offers a lot of information about cancer clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of cancer.

The next section helps explain the areas of research going on today about this type of cancer. Use the menu on the side of your screen to select Latest Research, or you can select another section, to continue reading this guide.

Rhabdomyosarcoma - Childhood - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 02/2014

ON THIS PAGE: You will read about the scientific research being done now to learn more about rhabdomyosarcoma and how to treat it. To see other pages, use the menu on the side of your screen.

Doctors are working to learn more about rhabdomyosarcoma, ways to prevent it, how to best treat it, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your child’s doctor about the diagnostic and treatment options best for your child.

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current rhabdomyosarcoma treatments in order to improve patients’ comfort and quality of life. For children with rhabdomyosarcoma at a low risk of recurrence, research is focused on decreasing the side effects of treatment by decreasing the total dose of chemotherapy, limiting radiation therapy, and decreasing the total length of time in treatment.

Higher-risk rhabdomyosarcoma. For children with higher risk or later stage disease, research is aimed at improving treatment outcome. An intermediate-risk study, started in 2006 and recently completed (mid-2013), tested whether the combination of irinotecan/vincristine, added to VAC chemotherapy, improves outcome. This study also tested whether the early use of local radiation therapy could decrease local recurrences. Results are now being analyzed. A new European study is testing whether the addition of doxorubicin will improve survival.

A recently completed COG study for metastatic rhabdomyosarcoma tested whether irinotecan, vincristine, and chemotherapy given every two weeks improves outcome. The early results of this study show that this combination therapy has the same amount of side effects during therapy as VAC used in prior studies for patients with high risk disease. The results also show that this therapy may improve survival for patients with embryonal disease. The longer term results are still being analyzed.

Targeted therapy. Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells while limiting damage to normal cells. Learn more about the basics of targeted treatments.

For patients with high-risk disease, current research is evaluating a type of targeted therapy that blocks the nutrients needed for a tumor to grow and spread. Monoclonal antibodies are a type of drug such as IMC-A12 (Cixutumumab) that may block tumor growth. Clinical trials are evaluating IMC-A12 with or without temozolamide (Temodar) in combination with the backbone chemotherapy recently studied for patients with high-risk disease. This ongoing study just completed enrollment.

A COG clinical trial recently evaluated two new drugs: bevacizumab (Avastin; a drug that blocks blood vessel growth) and temsirolimus (Torisel; a new anti-cancer drug) each in combination with the chemotherapy drugs vinorelbine (Navelbine) and cyclophosphamide (Cytoxan, Clafen, Neosar) for children with rhabdomyosarcoma that has recurred after initial therapy. This clinical trial is studying which drug, if either, is better in combination with vinorelbine and cyclophosphamide. This ongoing clinical trial just completed enrollment, and early results show a greater response rate from tumors in patients who received temsirolims in combination with vinorelbine and cyclophosphamide. Additional studies are being planned to determine if this early improved response rate with this combination could lead to greater survival rates for patients with rhabdomyosarcoma.

New approaches to treatment. Research to improve the understanding of rhabdomyosarcoma and how drugs affect this disease are also being performed. Investigations into the importance of gene translocations in rhabdomyosarcoma are underway. Identifying these translocations may be better than conventional methods of identification in distinguishing between the two major types of rhabdomyosarcoma. Research about new treatments, including the use of human rhabdomyosarcoma growing as a tumor xenograft (tissue taken from a healthy part of the body to replace unhealthy tissue in another part of the body), are ongoing.

Tissue collection. All children with rhabdomyosarcoma are encouraged to enroll on the COG study D9902. This study collects tumor and bone marrow samples that are not needed for the child’s medical care, so that scientists can learn more about the disease.

Looking for More About Latest Research?

If you would like additional information about the latest areas of research regarding rhabdomyosarcoma, explore these related items that take you outside of this guide:

  • To find clinical trials specific to your diagnosis, talk with your child’s doctor or search online clinical trial databases now.
  • Learn more about current COG studies for rhabdomyosarcoma.
  • Visit ASCO’s CancerProgress.Net website to learn more about the historical pace of research for childhood (pediatric) cancers. Please note this link takes you to a separate ASCO website.

The next section addresses how to cope with the symptoms of the disease or the side effects of its treatment. Use the menu on the side of your screen to select Coping with Side Effects, or you can select another section, to continue reading this guide.  

Rhabdomyosarcoma - Childhood - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 02/2014

ON THIS PAGE: You will find out more about steps to take to help cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.

Fear of treatment side effects is common after a diagnosis of cancer, but it may help to know that preventing and controlling side effects is a major focus of your child’s health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for rhabdomyosarcoma are described in detail within the Treatment Options section. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the cancer’s stage, the length and dosage of treatment(s), and your child’s overall health.

Before treatment begins, talk with your child’s doctor about possible side effects of each type of treatment your child will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving your child may need during treatment and recovery, as family members and friends often play an important role in the care of a person with rhabdomyosarcoma. Learn more about caregiving.

In addition to physical side effects, there may be emotional and social effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies, including concerns about managing the cost of your cancer care

During and after treatment, be sure to tell the health care team about the side effects your child experiences, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care. Learn more by reading the After Treatment section or talking with your child’s doctor.

The next section helps explain medical tests and check-ups needed after finishing cancer treatment. Use the menu on the side of your screen to select After Treatment, or you can select another section, to continue reading this guide.  

Rhabdomyosarcoma - Childhood - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 02/2014

ON THIS PAGE: You will read about your child’s medical care after cancer treatment is finished and why this follow-up care is important. To see other pages, use the menu on the side of your screen.

After treatment for rhabdomyosarcoma ends, talk with your child’s doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your child’s recovery for the coming months and years. All children treated for cancer, including rhabdomyosarcoma, should have life-long, follow-up care.

Children treated for rhabdomyosarcoma should be monitored for signs that the cancer has come back. If there is a recurrence, it is most common within the first three years after diagnosis. During this time, routine monitoring should include regular physical examinations and imaging studies at least every three to six months for the first two years after completing treatment.

Based on the type of treatment your child received, the doctor will also determine what examinations and tests are needed to check for long-term side effects of the treatment. Children should be routinely monitored for growth patterns, development of sexual maturity and fertility, and bladder function. If your child received radiation therapy to the eye or mouth, regular eye examinations and dental examinations are important. Children who had a tumor on an arm or leg may have decreased growth in the affected limb and differences in limb length as the child ages. This should be watched closely, and an evaluation by an orthopedist (bone doctor) is recommended if this develops. Long-term side effects also include the possibility of a secondary cancer, such as bone sarcoma, a brain tumor, or acute myeloid leukemia (AML). Your child’s doctor can recommend the necessary screening tests depending on the therapy your child received. Follow-up care should also address your child’s quality of life, including any developmental or emotional concerns. Learn more about childhood cancer survivorship.

Your family is encouraged to organize and keep a record of the child’s medical information. That way, as the child enters adulthood, it’s important that he or she has a clear, written history of the diagnosis, the treatment given, and the doctor’s recommendations about the schedule for follow-up care. The doctor’s office can help you create this. This information will be valuable to doctors who care for your child during his or her lifetime. ASCO offers cancer treatment summary forms to help keep track of the cancer treatment your child received and develop a survivorship care plan once treatment is completed.

Children who have had cancer can also enhance the quality of their future by following established guidelines for good health into and through adulthood, including not smoking, maintaining a healthy weight, eating a balanced diet, and participating in regular physical activity. Talk with the doctor about developing a plan that is best for your child’s needs. Learn more about the next steps to take in survivorship.

The next section offers a list of questions you may want to ask. Use the menu on the side of your screen to select Questions to Ask the Doctor, or you can select another section, to continue reading this guide.

Rhabdomyosarcoma - Childhood - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 02/2014

ON THIS PAGE: You will find some questions to ask your child’s doctor or other members of the health care team, to help you better understand your child’s diagnosis, treatment plan, and overall care. To see other pages, use the menu on the side of your screen.

Talking often with the doctor is important to make informed decisions about your child’s health care. These suggested questions are a starting point to help you learn more about your child’s cancer care and treatment. You are also encouraged to ask additional questions that are important to your family. You may want to print this list and bring it to your child’s next appointment, or download Cancer.Net’s free mobile app for an e-list and other interactive tools to manage his or her care.

  • Where exactly is the tumor located?
  • Are more tests needed to confirm the diagnosis of rhabdomyosarcoma?
  • How frequently do you treat patients with rhabdomyosarcoma?
  • Can you explain my child’s pathology report (laboratory test results) to me?
  • What group and stage is the tumor? What does this mean?
  • Is the histology of the tumor favorable or unfavorable? What does this mean?
  • What are my child’s treatment options?
  • What clinical trials are open to my child? Where are they located, and how do I find out more about them?
  • What treatment plan do you recommend? Why?
  • What is the goal of each treatment? Is it to eliminate the cancer, help my child feel better, or both?
  • Who will be part of my child’s health care team, and what does each member do?
  • Who will be coordinating my child’s overall treatment and follow-up care?
  • What are the possible side effects of this treatment, both in the short term and in the long term?
  • How will this treatment affect my child’s daily life? Will he or she be able to go to school and perform his or her usual activities?
  • Could this treatment affect my child’s ability to become pregnant or have children in the future? If so, should my family talk with a fertility specialist before cancer treatment begins?
  • If I’m worried about managing the costs related to my child’s cancer care, who can help me with these concerns?
  • What follow-up tests will my child need, and how often will he or she need them?
  • What support services are available to my child? To my family?
  • Whom should I call for questions or problems?

The next section offers some more resources that may be helpful to you. Use the menu on the side of your screen to select Additional Resources, or you can select another section, to continue reading this guide.

Rhabdomyosarcoma - Childhood - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 02/2014

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about cancer care and treatment. This is the final page of Cancer.Net’s Guide to Childhood Rhabdomyosarcoma. To go back and review other pages, use the menu on the side of your screen.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer, both for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond. Here are a few sections that may get you started in exploring the rest of Cancer.Net:

This is the end of Cancer.Net’s Guide to Childhood Rhabdomyosarcoma. Use the menu on the side of your screen to select another section to continue reading this guide.