Wilms' tumor staging: Difference between revisions
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==Staging== | ==Staging== | ||
Both the results of the imaging studies and the surgical and pathologic findings | * Both the results of the imaging studies and the surgical and pathologic findings post nephrectomy are used to determine the stage of disease. | ||
* The staging system was originally developed by the NWTS Group and is still used by the COG.<ref name="pmid2809467">{{cite journal |vauthors=Fujita H |title=Freeze-fracture method and its application to the cell biology |journal=J Electron Microsc (Tokyo) |volume=38 Suppl |issue= |pages=S110–7 |date=1989 |pmid=2809467 |doi= |url=}}</ref> | |||
===Stage I (43% of patients) === | ===Stage I (43% of patients) === |
Revision as of 15:46, 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
- Both the results of the imaging studies and the surgical and pathologic findings post nephrectomy are used to determine the stage of disease.
- The staging system was originally developed by the NWTS Group and is still used by the COG.[1]
Stage I (43% of patients)
For stage I Wilms' tumor, 1 or more of the following criteria must be met:
- Tumor is limited to the kidney and is completely excised.
- The surface of the renal capsule is intact.
- The tumor is not ruptured or biopsied (open or needle) prior to removal.
- No involvement of renal sinus vessels.
- No residual tumor apparent beyond the margins of excision.
Treatment: Nephrectomy + 18 weeks of chemotherapy
Outcome: 98% 4-year survival; 85% 4-year survival if anaplastic
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 III (23% of patients)
For Stage III Wilms' tumor, 1 or more of the following criteria must be met:
- Unresectable primary tumor.
- Lymph node metastasis.
- Positive surgical margins.
- Preoperative chemotherapy has been given.
- Tumor spillage involving peritoneal surfaces either before or during surgery, or transected tumor thrombus.
Treatment: Abdominal radiation + 24 weeks of chemotherapy + nephrectomy after tumor shrinkage
Outcome: 95% 4-year survival; 56% 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.