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Rhabdomyosarcoma: Frequently Asked Questions

Here are answers to some questions frequently asked by parents of children with rhabdomyosarcoma:

Like most tumors of childhood, the cause of rhabdomyosarcoma is not completely understood. Scientists believe that rhabdomyosarcoma tumors begin in primitive cells that normally turn into muscle cells. The tumor, which most often appears in early childhood, can occur essentially anywhere in the body.

Only in rare cases do children appear to inherit an increased risk for getting rhabdomyosarcoma as part of a genetic syndrome. Most of the time, there is no identifiable risk factor.

It’s rare for other family members to be affected. We always analyze family history for each new patient. Occasionally a genetic problem is suspected. In that case and if desired, the patient and family members have the opportunity to be evaluated in our Pediatric Familial Cancer to try to understand possible inherited risks that might predispose them to rhabdomyosarcoma or other cancers.

Once the diagnosis has been confirmed, your oncologist will assign a stage to the rhabdomyosarcoma. Staging for rhabdomyosarcoma is complex and depends on the location of the primary tumor, the size of the tumor, the involvement of nearby tissues, whether lymph nodes are involved, and if the cancer has spread to distant parts of the body. Rhabdomyosarcomas are also given a group number. The group number takes into account how much of the primary tumor has been removed and if the rhabdomyosarcoma cells have spread.

If the tumor cells have metastasized (spread), they can be found in a wide variety of locations in the body, including the lungs, brain, breast, bones, bone marrow, liver, lymph nodes, spleen and other soft tissue sites.

Rhabdomyosarcoma may require surgery for the initial biopsy, removal of all tumor tissue in the primary location, and (sometimes) resection in areas where it has spread. Sometimes the tumor is removed after treatment with chemotherapy and/or radiation. In some cases, the tumor cannot be removed. In these rare cases, it is treated with radiation and chemotherapy.

Chemotherapy, before and after surgery, is a critical part of the comprehensive and multidimensional treatment of rhabdomyosarcoma. It is sometimes given before surgery in order to shrink the tumor, which can make surgical removal easier to accomplish. Chemotherapy is also given after surgery, even if there is no other evidence of tumor left behind. This is done because we know that microscope amounts of rhabdomyosarcoma remain after surgery. Chemotherapy is used to eradicate these cells to minimize the chance that the cancer will come back at other sites. Chemotherapy for rhabdomyosarcoma is nearly always given intravenously (IV) through a catheter called a "central line." It is usually given over a period of 2 to 5 days in the hospital. Each of these 2 to 5 day “cycles” of chemotherapy is given every 2 – 3 weeks. Usually, about 14 cycles of chemotherapy are used.

Radiation is often given to sites where the rhabdomyosarcoma is visible. In situations where surgery cannot be used, radiation treatments can effectively control the tumor and prevent the cancer from growing. In some cases, radiation therapy is used after a primary tumor is removed in an effort to get rid of any cancer cells that might have been on the “margin” or edge of the tumor removal site.

Chemotherapy for rhabdomyosarcoma is usually given in cycles, with each cycle covering a period of 2 to 5 days in the hospital. A new cycle is given approximately every 3 weeks. For most of the time between cycles, patients only return to clinic for a check-up once per week. The number of cycles varies, depending on the stage and group number; the total duration of chemotherapy can be from 12 to 45 weeks. At some stage before or during the treatment, surgery is often performed to remove the visible tumor. The recovery period in the hospital varies from patient to patient, lasting from several days to 1 to 2 weeks. Radiation therapy, if needed, is given 5 days per week for 4 to 6 weeks. It is administered in the outpatient treatment center. Chemotherapy is often modified or delayed during surgery or radiation therapy.

Chemotherapy drugs not only attack the cancer cells, they can injure normal tissue and blood-forming cells. For this reason, your child may experience some of the common side effects of chemotherapy including low blood counts (causing anemia), nausea, vomiting, hair loss, mouth sores, irregular periods (girls) and inability to produce sperm (boys). Radiation therapy may put children at a risk of developing a second malignant tumor later in life. Your treatment team will do everything they can to reduce or prevent side effects of treatment.

Patients and their parents should talk to their doctor about the reproductive risks associated with cancer treatment. In certain situations, there are options available to try to preserve fertility, such as with sperm banking. These programs are available at the University of Chicago Medicine Comer Children’s Hospital.

Scientists and researchers are continually making progress in the fight against cancer. At any given time, many clinical trials of new drugs and protocols for childhood cancers are being tested. Across North America, most children with cancer are treated on a clinical research trial. Your physician only recommends a clinical trial when your care team believes it offers the best chance for a cure. Even if your treating physician recommends treatment on a clinical trial, you always have the option to choose the “standard of care” treatment that does not involve research.

The prognosis for survival varies widely depending on the Stage and Group of the rhabdomyosarcoma, and the type of treatment used. The survival rate for rhabdomyosarcoma patients who undergo chemotherapy and surgery, and who have no metastases, is more than 80 percent. Historically, survival rates for patients who have metastases are approximately 30 percent.

Unfortunately, rhabdomyosarcoma can return after treatment. It can come back at the original site or at virtually any other site in the body. Additional surgery, radiation, and chemotherapy or other experimental therapy can be used.

Chemotherapy for rhabdomyosarcoma is usually given in cycles, with each cycle covering a period of 2 to 5 days in the hospital. A new cycle is given approximately every 3 weeks. For most of the time between cycles, patients only return to clinic for a check-up once per week. The number of cycles varies, depending on the stage and group number; the total duration of chemotherapy can be from 12 to 45 weeks. At some stage before or during the treatment, surgery is often performed to remove the visible tumor. The recovery period in the hospital varies from patient to patient, lasting from several days to 1 to 2 weeks. Radiation therapy, if needed, is given 5 days per week for 4 to 6 weeks. It is administered in the outpatient treatment center. Chemotherapy is often modified or delayed during surgery or radiation therapy.

Behind the scenes, University of Chicago physicians and scientists are studying the biological and genetic basis of sarcomas and other cancers. Their findings may contribute to more effective ways to diagnose and treat rhabdomyosarcoma and to prevent the recurrence of this and other cancers that affect children and adults. Read more about sarcoma research under way at the University of Chicago.