Skip to content

Cancer Health Center

Font Size

Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Clear Cell Sarcoma of the Kidney

Because of the relative rarity of this tumor, all patients with clear cell sarcoma of the kidney should be considered for entry into a clinical trial. Treatment planning by a multidisciplinary team of cancer specialists (pediatric surgeon or pediatric urologist, pediatric radiation oncologist, and pediatric oncologist) with experience treating renal tumors is required to determine and implement optimum treatment.

Standard Treatment Options

Recommended Related to Cancer

Overview

This complementary and alternative medicine (CAM) information summary provides an overview of the use of Cannabis and its components as a treatment for people with cancer -related symptoms caused by the disease itself or its treatment. This summary contains the following key information: Cannabis has been used for medicinal purposes for thousands of years. By federal law, the possession of Cannabis, also known as marijuana, is illegal in the United States. The U.S. Food and Drug Administration...

Read the Overview article > >

The approach for treating clear cell sarcoma of the kidney is different from Wilms tumor since the OS of children with clear cell sarcoma of the kidney remains lower than for patients with favorable histology Wilms tumor. In the National Wilms Tumor Study-3 (NWTS-3), the addition of doxorubicin to the combination of vincristine, dactinomycin, and radiation therapy resulted in an improvement in disease-free survival for patients with clear cell sarcoma of the kidney.[1] The National Wilms Tumor Study-4 (NWTS-4) showed that patients treated with vincristine, doxorubicin, and dactinomycin for 15 months had an improved relapse-free survival compared with patients treated for 6 months (88% vs. 61% at 8 years).[2] In the National Wilms Tumor Study-5 (COG-Q9401) trial, children with stages I to IV clear cell sarcoma of the kidney were treated with a new chemotherapeutic regimen combining vincristine, doxorubicin, cyclophosphamide, and etoposide in an attempt to further improve the survival of these high-risk groups. All patients received radiation therapy to the tumor bed. With this treatment, the 5-year EFS was approximately 79% and OS was approximately 89%. Stage I patients had 100% 5-year EFS and OS. Stage II patients had a 5-year EFS of approximately 87% and OS of approximately 97%. Stage III patients had an approximately 74% 5-year EFS and an approximately 87% 5-year OS. Stage IV patients had a 5-year EFS of approximately 36% and 5-year OS of 45%. Clear cell sarcoma of the kidney has been characterized by late relapses; however, in NWTS-5, most relapses occurred within 3 years. In NWTS-5, the most common site of recurrence was the brain,[3] which has been successfully treated with combination chemotherapy, surgery, and radiation therapy.[4][Level of evidence: 2A]

  • Regimen DD-4A (vincristine, dactinomycin, and doxorubicin) for 15 months and radiation therapy.[2]
  • Regimen I (vincristine, doxorubicin, cyclophosphamide, and etoposide) and radiation therapy.[3]
    1|2
    Next Article:

    Today on WebMD

    Colorectal cancer cells
    A common one in both men and women.
    Lung cancer xray
    See it in pictures, plus read the facts.
     
    sauteed cherry tomatoes
    Fight cancer one plate at a time.
    Ovarian cancer illustration
    Do you know the symptoms?
     
    Jennifer Goodman Linn self-portrait
    Blog
    what is your cancer risk
    HEALTH CHECK
     
    colorectal cancer treatment advances
    Video
    breast cancer overview slideshow
    SLIDESHOW
     
    prostate cancer overview
    SLIDESHOW
    lung cancer overview slideshow
    SLIDESHOW
     
    ovarian cancer overview slideshow
    SLIDESHOW
    Actor Michael Douglas
    Article