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Treatment for Initially Non-Removable Tumors

Patients whose tumor could not be removed at the beginning of treatment will have a biopsy done first. After the biopsy, the patient will be treated with chemotherapy, followed by imaging studies and surgery to remove the remaining tumor. Chemotherapy and radiation therapy will be given after the tumor is removed as completely as possible.

One reason as to why the tumor may not be removed completely at the very beginning is because it could have spread into surrounding blood vessels, the abdominal wall, or other important structures like the spleen, pancreas, colon, or liver. It could cause more damage to try to completely remove the tumor in these cases.

Chemotherapy

Chemotherapy is given for 12 weeks after the biopsy. At week 13, the tumor is re-evaluated for possible surgery. Chemotherapy makes the removal of the tumor easier. In addition your doctor will look at the tumor under the microscope to check how well the chemotherapy is working.

While chemotherapy before surgery helps control the spread of the tumor, chemotherapy after surgery is also critical in order to treat any tumor cells left after surgery.

Chemotherapy is determined by your child's tumor stage. Two protocol regimens are used to treat clear cell sarcoma:

Patients with stage I-IV CCSK are most commonly treated with Regimen I.
Chemotherapy drugs used are cyclophosphamide, etoposide, vincristine, and doxorubicin.

Patients with stage IV CCSK can choose to participate in the clinical trial Regimen UH1.
Chemotherapy drugs used are cyclophosphamide, etoposide, vincristine, and doxorubicin as well as carboplatin.

Clinical trials offer the most up-to-date chemotherapy treatments with the best-known results for improvement or cure of clear cell sarcoma. The majority of young cancer patients decide to enter into a Phase III clinical trial such as the one offered by the Children's Oncology Group.

More about the newest clinical trials.

Surgery

Surgery to remove the tumor should happen as soon as it can safely be done. In most cases, this will be after more imaging studies have been done to evaluate the tumor during week 13. It should be done by a pediatric surgeon or pediatric urologist that is used to taking care of children with cancer. After the surgeon has checked the whole abdomen for any additional signs of tumor, the whole kidney including the tumor is usually removed. In addition, the surgeon will take out some lymph nodes to check if any tumor is present in these lymph nodes.

More information about surgical treatments.

Radiation

Radiation therapy will be given after the tumor is removed as completely as possible. Usually flank radiation is given, however in some cases, whole abdominal radiation might be required.

Dosage and more information about radiation therapy.