Skip to content

Cancer Health Center

Font Size

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

Because of the relative rarity of this tumor, all patients with rhabdoid tumor 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.

Patients with rhabdoid tumors of the kidney continue to have a poor prognosis with 4-year overall survival (OS) rates of 42% for stages I and II (n = 40) and 16% for stages III, IV, and V (n = 102).[1]

Recommended Related to Cancer

General Information About Renal Cell Cancer

Incidence and Mortality Estimated new cases and deaths from renal cell (kidney and renal pelvis) cancer in the United States in 2013:[1] New cases: 65,150. Deaths: 13,680. Renal cell cancer, also called renal adenocarcinoma, or hypernephroma, can often be cured if it is diagnosed and treated when still localized to the kidney and to the immediately surrounding tissue. The probability of cure is directly related to the stage or degree of tumor dissemination. Even when regional lymphatics...

Read the General Information About Renal Cell Cancer article > >

Standard Treatment Options

  • No satisfactory treatment has been developed for these children.[2] The National Wilms Tumor Study (NWTS)-5 (COG-Q9401) closed the treatment arm for rhabdoid tumor with cyclophosphamide, etoposide, and carboplatin because poor outcome was observed. Combinations of etoposide and cisplatin; etoposide and ifosfamide; and ifosfamide, carboplatin, and etoposide (ICE chemotherapy) have been used (COG-Q9401).[3,4] In a review of 142 patients from NWTS-1 through NWTS-5, stage and age are significant prognostic factors. Patients with stage I and stage II disease had an OS rate of 42%; higher stage was associated with a 16% OS. Infants younger than 6 months at diagnosis demonstrated a 4-year OS of 9%, whereas OS in patients aged 2 years and older was 41%. All except one patient with a central nervous system lesion died.[1]
  • The Société Internationale d'Oncologie Pédiatrique (SIOP) renal tumor group has noted that preoperative chemotherapy does not seem to translate into improved survival. Delays in surgery lead to worse survival compared with patients treated according to direct surgery strategies.[5]

Treatment Options Under Clinical Evaluation

The following treatment option is currently under investigation in a Children's Oncology Group (COG) clinical trial. Information about ongoing clinical trials is available from the NCI Web site.

  • COG-AREN0321 (Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors): In this trial, patients with stages I, II, III, and IV (without measurable disease) will be treated with UH-1 (which consists of cyclophosphamide, carboplatin, and etoposide alternating with vincristine, doxorubicin, and cyclophosphamide for 30 weeks) and radiation therapy.
    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
    Real Cancer Perspectives
     
    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