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The University of Chicago Medicine - Comer Children's Hospital

Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS): Frequently Asked Questions

Most of the time, the cause of an individual patient’s non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) is not known. Certain types of NRSTS are thought to occur at a site where radiation therapy was previously given. Certain types of NRSTS can also develop in children or young adults who have an inherited risk to developing cancer.

Children with certain hereditary conditions are at an increased risk for developing the disease. It has also been associated with radiation therapy given earlier in life for other types of cancer.

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, the patient and certain family members will be offered the chance to be evaluated in our Pediatric Familial Cancer to better understand whether inherited trait that puts them at risk to develop cancer.

Once the diagnosis has been confirmed, your oncologist will assign a stage to the NRSTS. Staging for NRSTS 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.

The tumor cells can invade surrounding tissue and metastasize (spread) to other organs in the body. When NRSTS spreads, it most commonly found in the lungs. Some types of NRSTS can spread to lymph nodes or other sites.

NRSTS usually requires surgery for the initial biopsy, removal of all or part of the primary tumor, and removal of tumor that has metastasized (spread) to other sites. Surgery is the primary treatment for NRSTS

Chemotherapy can be a critical part of the comprehensive and multidimensional treatment for some NRSTS, especially those that are at high-risk of recurrence and/or have spread to other parts of the body. Chemotherapy is given intravenously (IV) through a catheter (called a “central line”) that is placed under the skin. Chemotherapy is usually given over a period of 2 to 3 days in the hospital. Each of these 2 – 5 day “cycles” of chemotherapy is given about every 3 weeks, for approximately 6 cycles. For some types of NRSTS, chemotherapy is not used.

Radiation is often used in patients with NRSTS to help shrink the tumor before and/or after surgery. In some cases, it is used instead of surgery.

When chemotherapy is used for NRSTS, it is usually given in cycles, with each cycle covering a period of 2 to 3 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 type of NRSTS and the stage; if chemotherapy is used, the total duration of chemotherapy is usually 15 to 20 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 depending on the exact type of NRSTS, whether the tumor has a low- or high-grade, and whether it is at an early or advanced stage when it is detected and treated. For patients with low-grade disease that has not metastasized (spread), the survival rate nears 90%. Historically, about 15% or less of the patients with high-grade, metastatic NRSTS are cured.

Unfortunately, non-rhabdomyosarcoma can come back after treatment, even when it has been in remission for several years. In these cases, the recurrent NRSTS can often be treated using chemotherapy, radiation and surgery, just like the original tumor. In addition, newer experimental medicines may also be available.

When chemotherapy is used for NRSTS, it is usually given in cycles, with each cycle covering a period of 2 to 3 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 type of NRSTS and the stage; if chemotherapy is used, the total duration of chemotherapy is usually 15 to 20 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 non-rhabdomyosarcomas 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.