Pediatric Sarcoma

Pediatric sarcoma is a cancer that forms in the supportive tissues (bone), soft tissue (muscle) or connective tissues (tendons). Called pediatric sarcoma because it is primarily found in children, it also can occur in adolescents and young adults.

Most pediatric sarcomas are one of three types: rhabdomyosarcoma (the most common), Ewing sarcoma or osteosarcoma. Following are descriptions of each type, along with staging and typical treatment information.


Rhabdomyosarcoma (RMS) forms in immature cells that normally become muscle. It develops in a type of muscle called striated muscle. Striated muscles are the skeletal voluntary muscles, which are the muscles of the arms, legs, and rest of the body that people can control. RMS is often widely spread by the time a diagnosis is made. RMS is slightly more common in boys than in girls and is most common in children under the age of five.

There are three types of RMS. Embryonal is the most common type and occurs most often in the head and neck area or in the genital or urinary organs. Alveolar occurs most often in the arms or legs, chest, abdomen, genital organs or anal area. Anaplastic rarely occurs in children. Other less common types of sarcomas can form in tendons, nerves or blood vessels.

Staging for RMS, which helps doctors plan treatment, involves a staging system, a grouping system and a risk group:

  • The staging system considers the size of the tumor, where it is in the body and if it has spread to other parts of the body. Each tumor is classified as “favorable” or “unfavorable” based on what the cells look like under a microscope (known as histology). 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.
  • The grouping system is based on whether the cancer has spread and whether all the cancer was removed by surgery.
  • The risk group is based on the staging system and the grouping system and describes the chance that rhabdomyosarcoma will recur (come back). RMS is then labeled low-risk, intermediate-risk or high-risk childhood RMS.

Treatment for RMS generally consists of surgery and combination chemotherapy (two or more chemotherapy drugs used together). Every child treated for RMS should receive chemotherapy to decrease the chance cancer will recur. The type of drug, dose and the number of treatments given depends on whether the child has low-risk, intermediate-risk or high-risk RMS.

Ewing Sarcoma

Ewing sarcoma forms in the bone or soft tissues when healthy cells in the bone change and grow uncontrollably, forming a mass called a tumor. Ewing sarcoma affects the bones or nearby soft tissue, most often developing in the leg, pelvis, rib, arm or spine. It also can occur in soft tissues. Ewing sarcoma can grow and spread to other parts of the body. It most often develops in children and young adults between the ages of 10 and 20, is more common in girls than boys and is rare among African and African-American individuals.

Although there is no official staging system for Ewing sarcoma, the following criteria help doctors describe Ewing sarcoma and plan the best treatments.

  • Localized Ewing sarcoma. The tumor is only found in the bone where it began or has just spread to nearby tissues.
  • Metastatic Ewing sarcoma. The tumor has spread from the bone where it began to another part of the body, such as the lungs, other bones or bone marrow. Rarely, the disease spreads to the lymph nodes, brain or spinal cord. Approximately 25 percent of children and teens with Ewing sarcoma will have obvious evidence of tumor spread at the time of diagnosis. Whether the tumor has spread is the most important factor used to determine treatment options and prognosis.
  • Recurrent Ewing sarcoma. Recurrent Ewing sarcoma is a tumor that has come back after treatment. If the cancer returns, another round of tests, often similar to those done at the time of the original diagnosis, will be done to learn about the extent of the recurrence.

Multi-drug chemotherapy is the primary treatment in combination with surgery and radiation therapy.


Osteosarcoma forms in the bone and it destroys tissue and weakens the bone. The disease begins when immature bone cells become cancer cells instead of developing into bone. The most common place for osteosarcoma to start is in the bones around the knee joint, either at the femur, which is the lower end of the thigh bone, or the tibia, which is the upper end of the shin bone. Another common place for osteosarcoma to start is the humerus, the upper arm bone near the shoulder. However, osteosarcoma can develop in any bone in the body. Rarely, osteosarcoma occurs as a tumor in the soft tissue of the body, outside the bone. It most often affects teens and young adults in their 20s.

Doctors use these terms to describe the stages of osteosarcoma and plan treatment.

  • Localized. The tumor is only in the bone where it began and in the tissue around it. The tumor has not spread to other parts of the body.
  • Metastatic. The tumor has spread from the bone where it began to another part of the body. Most often, it has spread to the lungs or other bones.
  • Recurrent. Recurrent osteosarcoma is a tumor that has come back during or after treatment. It can come back in the same place where it started or in another part of the body. Osteosarcoma recurs most often in the lungs and other bones. If there is a recurrence, the cancer may need to be staged again. This is called re-staging.

Surgery is the primary treatment for osteosarcoma.

Important treatment considerations

It is typically recommended that treatment begin right away because of how aggressive pediatric sarcoma can be. As you make these timely treatment decisions, also think about the following items.

  • A second opinion. Getting another opinion from a pediatric oncologist (preferably one who is experienced in treating sarcoma) will either confirm the original diagnosis and treatment recommendations or offer new information to consider.
  • Clinical trials. Clinical trials are research studies that explore whether a treatment is safe and effective and which strategies work best for certain illnesses or groups of people. Clinical trials may offer your child access to promising new treatments that aren’t available outside of the trial. It may comfort you to know that your child will receive high-quality care and be closely monitored throughout the trial. And, by simply participating, your child will help others who will need cancer treatment in the future. Clinical trials that are exploring innovative strategies using immunotherapies and targeted therapies for children are currently underway. Ask your doctor if clinical trials are an option for your child.
  • Treatment location. Most children with cancer receive treatment or participate in clinical trials at places that specialize in treating cancer in children, such as a children’s hospital, university medical center or cancer center. If you choose one of these facilities, you may have to travel for treatment. Staying in a hotel for an extended time can be very costly. Some hospitals and cancer centers offer free or reduced cost accommodations for children in treatment and their families, or they may be able to refer you to nearby lodging. Or, you may opt for treatment at a treatment center for adults that is near your home. There are pros and cons to both. Talk with your pediatric oncologist and advocacy organizations to learn your options (see Financial Resources).



Nutrition During Treatment


Eating the right kinds of foods before, during and after treatment can help your child feel better and possibly even have more tolerance for treatment. Talk to a member of your child’s health care team to help identify nutrition goals and plan ways to help your child meet them.

Loss of appetite is one common challenge. If you are concerned your child isn’t eating enough, talk with your doctor and try these tips.

Serve small meals and snacks throughout the day, rather than three large meals. Good snacks are peanut butter and crackers, cheese sticks, pudding, and cereal and milk.

Let your child eat whenever he or she feels hungry. Include high-calorie, high-protein foods, such as hamburgers, fries, pizza and ice cream. Fat is a rich source of energy and can be helpful when your child has trouble taking in enough calories.

Mix up the menu. Have pizza for breakfast or pancakes for dinner.

Try to get your child to drink most fluids between meals. Drinking fluid with meals can make your child feel too full.

Use colorful cups and straws to encourage drinking fluids.

Get creative. Use cookie cutters to cut shapes from sandwiches, gelatin, meats and cheeses. Make faces out of fruits and vegetables.

Have picnics. Spread a blanket on the living room floor or in the backyard.

Encourage your child to be physically active. Activity may make him or her want to eat.




Tips for Helping Your Child Adjust


Cancer treatment brings with it many emotional and physical changes. Talking about these changes before they happen, and how you will manage them together, may make treatment easier to handle.

Hair loss. If treatment will cause hair thinning or loss, let your child pick out ball caps, funky hats, scarves or wigs ahead of time. Encourage your child to wear them and get comfortable in them before they become necessary.

Body weight and self image. Some treatments, such as steroids, can cause weight gain. Other treatments may make it difficult to eat, resulting in weight loss. Help your child pick out cute, comfy clothes to accommodate any weight change. Feeling good about his or her appearance can make a tremendous difference in attitude.

Awkward moments. People may stare, mistake your child's gender or ask questions that are too personal. Even when the intent is not to hurt your child’s feelings, it may. Make a plan for how your child can respond to (or ignore) those moments. Humor is often a great way to keep the mood light.

Bad moods. Think of creative ways to boost your child’s spirits, such as shopping, books, movies or games. If you notice that feelings of sadness become frequent and long-lasting, your child may be depressed. If you notice signs of depression, talk with the doctor.

Friendships. Encourage your child to stay connected with old friends and to keep up with new friends met during treatment. Phone calls, texts, video chats and social media platforms make staying in touch easy, especially when an in-person visit may not be feasible. Let your child know that some children (and some adults) don’t always know how to act when a close friend becomes ill. It may help for your child to reach out first to keep things “normal.”

Hobbies. Explore new hobbies, such as music, art, reading or writing, that can be done anywhere, such as during hospital stays or at home when visitors aren’t recommended.


Additional Resources


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