Wilms' tumor staging: Difference between revisions
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Outcome: 96% 4-year survival; 70% 4-year survival if anaplastic | Outcome: 96% 4-year survival; 70% 4-year survival if anaplastic | ||
===Stage IV (10% of patients) === | ===Stage IV (10% of patients) === |
Revision as of 17:03, 3 June 2018
Wilms' tumor Microchapters |
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Wilms' tumor staging On the Web |
American Roentgen Ray Society Images of Wilms' tumor staging |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
There are 5 stages of wilms' tumor based on both the results of the imaging studies and the surgical and pathologic findings at nephrectomy.
Staging
- Staging of wilms tumor is done based upon:
- Extent of tumor anatomically.
- It is not done on the basis of its genetics, histology or molecular markers.
- Extensive disease and worse prognosis is denoted by a higher stage.
- Patients suffering from a higher stage tumor get more aggressive treatment modalities.
- The staging systems that are currently used are:[1]
- National Wilms Tumor Study (NWTS) system.
- International Society of Pediatric Oncology (SIOP) system.
Stage II (23% of patients)
For Stage II Wilms' tumor, 1 or more of the following criteria must be met:
- Tumor extends beyond the kidney but is completely excised.
- No residual tumor apparent at or beyond the margins of excision.
- Any of the following conditions may also exist:
- Tumor involvement of the blood vessels of the renal sinus and/or outside the renal parenchyma.
- The tumor has been biopsied prior to removal or there is local spillage of tumor during surgery, confined to the flank.
Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy
Outcome: 96% 4-year survival; 70% 4-year survival if anaplastic
Stage IV (10% of patients)
Stage IV Wilms' tumor is defined as the presence of hematogenous metastases (lung, liver, bone, or brain), or lymph node metastases outside the abdominopelvic region.
Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy + radiation of metastatic site as appropriate
Outcome: 90% 4-year survival; 17% 4-year survival if anaplastic
Stage V (5% of patients)
Stage V Wilms’ tumor is defined as bilateral renal involvement at the time of initial diagnosis. Note: For patients with bilateral involvement, an attempt should be made to stage each side according to the above criteria (stage I to III) on the basis of extent of disease prior to biopsy. The 4-year survival was 94% for those patients whose most advanced lesion was stage I or stage II; 76% for those whose most advanced lesion was stage III.
Treatment: Individualized therapy based on tumor burden
Stage I-IV Anaplasia
Children with stage I anaplastic tumors have an excellent prognosis (80-90% five-year survival). They can be managed with the same regimen given to stage I favorable histology patients.
Children with stage II through stage IV diffuse anaplasia, however, represent a higher-risk group. These tumors are more resistant to the chemotherapy traditionally used in children with Wilms’ tumor (favorable histology), and require more aggressive regimens.