Sarcoma

Pediatric Sarcoma

Pediatric sarcoma is a cancer that forms in the bone or soft tissues of the body. It is called pediatric sarcoma because it is primarily found in children, but it can also occur in adults.

Most pediatric sarcomas are one of three types. Following are descriptions of each of these types, along with staging and typical treatment information.

Rhabdomyosarcoma

Rhabdomyosarcoma forms in immature cells that normally become striated muscle. This type of muscle is skeletal voluntary muscle, which makes up the muscles that people can control, such as those of the arms and legs. Rhabdomyosarcoma has often spread beyond the original site by the time a diagnosis is made. This type of pediatric sarcoma is slightly more common in boys than in girls and is most common in children under the age of five.

There are three subtypes of rhabdomyosarcoma. Embryonal is the most common 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 sarcoma can form in tendons, nerves or blood vessels.

Staging for rhabdomyosarcoma, which helps doctors plan treatment, involves the following.

  • 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 assigned after the staging grouping systems are determined and is a description of the chance that rhabdomyosarcoma will recur (come back). The risk groups are low-risk, intermediate-risk and high-risk childhood rhabdomyosarcoma.

Treatment for rhabdomyosarcoma generally consists of surgery and combination chemotherapy (two or more chemotherapy drugs used together). Every child treated for this sarcoma should receive chemotherapy to decrease the chance cancer will recur. The type of drug, dose and the number of treatments given depend on the risk level.

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. It 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 individuals of African descent.

Although there is no official staging system for Ewing sarcoma, doctors consider the following criteria to describe and plan treatment.

  • Localized Ewing sarcoma. The tumor is found only in the bone where it began or has spread only 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. Whether the tumor has spread is the most important factor used to determine treatment options and prognosis.
  • Recurrent Ewing sarcoma. If the cancer returns, it is called recurrent Ewing sarcoma. 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.

For Ewing sarcoma, multidrug chemotherapy is the primary treatment in combination with surgery and radiation therapy.

Osteosarcoma

Osteosarcoma can develop in any bone in the body. It destroys tissue and weakens the bone. The disease begins when immature bone cells become cancer cells instead of developing into mature bone cells. 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. 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 twenties.

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. It 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, osteosarcoma spreads 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 issues.

A second 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 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. Ask your doctor if a clinical trial is an option for your child (see Clinical Trials).

Treatment location must be considered because 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. You may choose a treatment center for adults near your home. There are pros and cons to both. Talk with your pediatric oncologist and advocacy organizations to learn about your options (see Resources).

 

 

Helping Your Child Cope During Treatment

Continuing to parent as you did before your child was diagnosed with sarcoma may seem like an extraordinary task, but keep in mind that to your child, you are still the same mom or dad — cancer or not. Although you may feel overwhelmed at times, it helps to stay in a normal routine as much as possible. When the sarcoma or its treatments make you veer from “normal” life, switch gears, keeping in mind that flexibility, patience and honest communication are very important.

Most children, regardless of age, have challenges with nutrition during treatment, including a loss of appetite. That is understandable. When you don’t feel well, especially if you’re nauseated, food doesn’t sound good. Good nutrition is not only important for a growing body, eating right can help your child feel healthier and possibly even tolerate treatment better.

There are many ways you can encourage your child to eat. First, however, you must realize that your traditional meals and mealtimes may go by the wayside for the time being, and that’s okay. Your child should eat whenever he or she is hungry. Offer small meals and snacks throughout the day, rather than three large meals. Peanut butter and crackers, cheese sticks, pudding, and cereal with milk are good options. The body uses a lot of calories when it is healing, so include high-calorie and high-protein foods, such as hamburgers, fries, pizza and ice cream, if your child wants them. Although those may not seem like healthy choices, fat is a rich source of energy and can be helpful when your child has trouble taking in enough calories.

For young children, getting creative with your food and dinnerware can help. Use cookie cutters to cut shapes from sandwiches, gelatin, meats and cheeses. Make faces out of fruits and vegetables. Use colorful cups and straws to encourage drinking fluids. Sometimes drinking with meals may make your child too full to eat, so encourage fluids between meals.

Mixing up the menu may appeal to older kids. Serve pizza for breakfast or pancakes for dinner. A change of scenery can be fun, too. Have picnics by spreading a blanket on the living room floor or eating in the backyard. Encourage physical activity with the doctor’s approval because it may increase your child’s appetite.

A cancer diagnosis is a lot to handle and will affect your family emotionally. Think of creative ways to boost your child’s spirits, such as shopping, books, movies or games. Explore new hobbies that can be done during hospital stays or at home when visitors aren’t recommended, such as music, art, reading or writing. If you notice your child is frequently sad or appears to be depressed, call the doctor.

A teen’s friends are often a lifeline, and your child may find it easier to confide in a friend instead of you or another family member. Find ways to encourage continued friendships, including staying connected with old friends and making new ones 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 possible.

The physical side effects of treatment may be upsetting, too, especially if your child is at a sensitive age. Teens often put a lot of value in appearance and may find some of the physical changes, such as hair loss, embarrassing. Ask your child’s doctor if you should expect hair loss. If so, let your child pick out ball caps, hats, scarves or wigs ahead of time. Encourage your child to wear them and get used to them before they become necessary. Another physical change has to do with body 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, comfortable clothes to accommodate any weight change. Feeling good about his or her appearance can make a tremendous difference in attitude.

Let your child know that some children (and even some adults) don’t always know how to act when a close friend becomes ill. People may stare, mistake your child's gender or ask questions that are too personal. Even when there is no intent 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.

Lastly, lead by example. Show your child that you’re positive about the road ahead. Most important, trust your instincts. After all, you know your child best. And, remember, you’re not alone. Your child’s medical team is available to assist or recommend others who can.

 

 

Additional Resources

 

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