•Patients with stage I who undergo lymph node sampling do not undergo radiation therapy to the tumor bed.

•Any patient with stage I who has not undergone lymph node sampling is upstaged to stage II.

•Patients with stage IV undergo treatment with irinotecan and vincristine in an upfront window approach before treatment with cyclophosphamide, etoposide, vincristine, doxorubicin, and cyclophosphamide.

•Surgical treatment:

–Radical nephrectomy is the initial treatment of choice if the lesion is resectable.

–If the tumor is not resectable, a biopsy is performed, and chemotherapy is administered, followed by surgical resection after a response has been obtained.

–Patients with clear cell sarcoma of the kidney (CCSK) are treated with combination chemotherapy.

–The addition of doxorubicin to chemotherapeutic regimens has been shown to improve disease-free survival rates.

•After completing chemotherapy, patients should continue to have regular follow up with blood and radiographic evaluations on an outpatient basis.

•The follow up should be every 1–3 months for the first year, every 3–6 months for the second and third years, then yearly thereafter.

•There is an increase in number of CNS recurrences of clear cell sarcoma of the kidney (CCSK) which should be taken in consideration during follow ups.

•Clear cell sarcoma of the kidney tumors are associated with late recurrence; the most common site of recurrence is the brain and then the lung.

•The patients remain at risk for recurrence after 2 years posttherapy. Tumors may recur as long as 10 years after completion of treatment.

•Treatment of patients with recurrent clear cell sarcoma of the kidney:

–This depends on the initial chemotherapy.

–Cyclophosphamide and carboplatin should be considered if not used initially.

–Patients with recurrent clear cell sarcoma of the kidney that involves the brain can be treated with ifosfamide, carboplatin, and etoposide (ICE), coupled with local control consisting of either surgical resection and/ or radiation.

•Patients require follow-up evaluation for toxic effects resulting from chemotherapy, radiotherapy, or both. These include:

–Cardiomyopathy

–Patients are at risk for renal failure because they have a single kidney

–Radiation effects including asymmetry of the back muscles

–Secondary malignant neoplasms

–Infertility

–Risk of intestinal obstruction

4.4.7Prognosis

•The prognosis for CCSK has improved with the addition of doxorubicin to chemotherapy regimens with a 66 % reduction in overall mortality.

•This is particularly so for low stage tumors,

•The reported stage-dependent 6-year survival is:

–97 % for stage I tumors.

–75 % for stage II tumors.

–77 % for stage III tumors.

–50 % for stage IV tumors.

•Twenty-nine percent of patients with CCSK have lymph node metastases at the time of diagnosis, and bone metastasis is the most common form of relapse.

•Metastatic lesions have also been reported in the liver, brain, soft tissue sites, and lung with more unusual metastases to the skeletal muscles, testis, and salivary gland.

•Approximately 20 % of documented clear cell sarcoma of the kidney metastases occurred at least 3 years after initial diagno-

sis; bur relapses of CCSK as many as 10 years after original diagnosis have been reported.