Wilms' tumor staging: Difference between revisions
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==Overview== | ==Overview== | ||
Staging is determined by combination of imaging studies, and | [[Staging]] is determined by combination of imaging studies, and [[pathological]] findings if the tumor is operable (adapted from www.cancer.gov). Treatment strategy is determined by the stage: | ||
==Staging== | ==Staging== | ||
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For stage I Wilms' tumor, 1 or more of the following criteria must be met: | 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. | * Tumor is limited to the kidney and is completely excised. | ||
* The surface of the renal capsule is intact. | * The surface of the renal [[capsule]] is intact. | ||
* The tumor is not ruptured or biopsied (open or needle) prior to removal. | * The tumor is not ruptured or biopsied (open or needle) prior to removal. | ||
* No involvement of renal sinus vessels. | * No involvement of renal [[sinus]] vessels. | ||
* No residual tumor apparent beyond the margins of excision. | * No residual tumor apparent beyond the margins of [[excision]]. | ||
Treatment: [[Nephrectomy]] + 18 weeks of [[chemotherapy]] | Treatment: [[Nephrectomy]] + 18 weeks of [[chemotherapy]] | ||
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* No residual tumor apparent at or beyond the margins of excision. | * No residual tumor apparent at or beyond the margins of excision. | ||
* Any of the following conditions may also exist: | * Any of the following conditions may also exist: | ||
** Tumor involvement of the blood vessels of the renal sinus and/or outside the renal parenchyma. | ** 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. | ** 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 | Treatment: Nephrectomy + abdominal [[radiation]] + 24 weeks of chemotherapy | ||
Outcome: 96% 4-year survival; 70% 4-year survival if anaplastic | Outcome: 96% 4-year survival; 70% 4-year survival if anaplastic | ||
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===Stage III (23% of patients)=== | ===Stage III (23% of patients)=== | ||
For Stage III Wilms' tumor, 1 or more of the following criteria must be met: | For Stage III Wilms' tumor, 1 or more of the following criteria must be met: | ||
* Unresectable primary tumor. | * [[Unresectable]] primary tumor. | ||
* Lymph node metastasis. | * Lymph node [[metastasis]]. | ||
* Positive surgical margins. | * Positive surgical margins. | ||
* Tumor spillage involving peritoneal surfaces either before or during surgery, or transected tumor thrombus. | * [[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 | Treatment: Abdominal radiation + 24 weeks of chemotherapy + nephrectomy after tumor shrinkage | ||
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===Stage IV (10% of patients) === | ===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 | 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 | Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy + radiation of metastatic site as appropriate | ||
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===Stage V (5% of patients) === | ===Stage V (5% of patients) === | ||
Stage V Wilms’ tumor is defined as bilateral renal involvement at the time of initial diagnosis. | 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. | 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 | Treatment: Individualized therapy based on tumor burden | ||
===Stage I-IV Anaplasia=== | ===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 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. | 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. | ||
Revision as of 13:38, 17 September 2012
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Wilms' tumor staging On the Web |
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American Roentgen Ray Society Images of Wilms' tumor staging |
Overview
Staging is determined by combination of imaging studies, and pathological findings if the tumor is operable (adapted from www.cancer.gov). Treatment strategy is determined by the stage:
Staging
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.
- 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.