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==Overview==
==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 of wilms tumor is done based upon the extent of tumor [[Anatomy|anatomically]] and not done on the basis of its [[genetics]], [[histology]] or [[molecular marker]]<nowiki/>s. Extensive disease and worse [[prognosis]] is denoted by a higher stage. The staging systems that are currently used are National Wilms Tumor Study (NWTS) system and International Society of Pediatric Oncology (SIOP) system. Both these systems divide wilms tumor into 5 stages.  


==Staging==
==Staging==
Both the results of the imaging studies and the surgical and pathologic findings at 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.  
* Staging of wilms tumor is done based upon:
** Extent of [[Tumor cell|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:<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>
**  National Wilms Tumor Study (NWTS) system.
**  International Society of Pediatric Oncology (SIOP) system.


===Stage I (43% of patients) ===
=== NWTS system ===
For stage I Wilms' tumor, 1 or more of the following criteria must be met:
* This system is used throughout United States and Canada.
* Tumor is limited to the kidney and is completely excised.
* It is based on the evaluations done during surgery before the administration of surgery.
* The surface of the renal [[capsule]] is intact.
* According to this system wilms tumor is divided into following stages.<ref name="pmid16314292">{{cite journal |vauthors=Metzger ML, Dome JS |title=Current therapy for Wilms' tumor |journal=Oncologist |volume=10 |issue=10 |pages=815–26 |date=2005 |pmid=16314292 |doi=10.1634/theoncologist.10-10-815 |url=}}</ref>
* The tumor is not ruptured or biopsied (open or needle) prior to removal.
** '''Stage 1'''
* No involvement of renal [[sinus]] vessels.
*** Tumor is limited only to the [[kidney]].
* No residual tumor apparent beyond the margins of [[excision]].
*** When the tumor is resected the [[capsule]] is intact.
*** Resected tumor shows no evidence of involvement of renal sinus vessel.
** '''Stage 2'''
*** Tumor is extended beyond the [[kidney]], but can be resected completely.  
*** Tumor margins go beyond the renal capsule.
*** It extends into the renal capsule, soft tissues and blood vessels beyond the renal [[Parenchymal|parenchyma]].
*** But all this is within the margins of the resected tumor.
** '''Stage 3'''
*** Complete tumor cannot be resected.  
*** The remaining tumor is confined within the [[abdomen]].
*** Residual tumor remaining within abdomen includes :
**** Regional [[lymph nodes]]
**** [[Peritoneal]] surface
**** Incomplete resection of tumor
**** Tumor spillage
** '''Stage 4'''
*** In stage 4 either [[Metastasis|hematogenous metastasis]] are present or [[lymph nodes]] are affected beyond the abdominopelvic region.
*** [[Metastasis|Hematogenous metastasis]] include sites like:
**** [[Lung]]
**** [[Liver]]
**** [[Bone]]
**** [[Brain]]
** '''Stage 5'''
*** When Wilms tumor is diagnosed, bilateral renal involvement is seen.
*** Each kidney has to be staged separately.


Treatment: [[Nephrectomy]] + 18 weeks of [[chemotherapy]]
=== SIOP staging ===
* This system is followed in Europe.
Outcome: 98% 4-year survival; 85% 4-year survival if [[anaplastic]]
* This system is based on the surgical evaluation done after the size of the tumor is reduced after [[chemotherapy]].
 
* According to this system wilms tumor is divided into following stages.
===Stage II (23% of patients)===
** '''Stage 1'''
For Stage II Wilms' tumor, 1 or more of the following criteria must be met:
*** Tumor is limited only to the kidney.
* Tumor extends beyond the kidney but is completely excised.
*** If the tumor extends beyond the kidney, a fibrous pseudocapsule surrounds it.
* No residual tumor apparent at or beyond the margins of excision.
*** Tumor can be found in the pelvic system but should not involve ureter walls.
* Any of the following conditions may also exist:
** '''Stage 2'''  
** Tumor involvement of the blood vessels of the renal sinus and/or outside the renal [[parenchyma]].
*** Tumor is extended beyond the kidney, but can be resected completely.
** The tumor has been biopsied prior to removal or there is local spillage of tumor during surgery, confined to the flank.
*** After resection there is no evidence beyond the resection margins.
 
** '''Stage 3'''
Treatment: Nephrectomy + abdominal [[radiation]] + 24 weeks of chemotherapy
*** After tumor is resected surgically, some residual [[tumor]] still remains.  
 
*** This remainder tumor is confined to the [[abdomen]].
Outcome: 96% 4-year survival; 70% 4-year survival if anaplastic
*** No hematogenous metastasis is present.
 
** '''Stage 4'''
===Stage III (23% of patients)===
*** Hematogenous spread os positive.
For Stage III Wilms' tumor, 1 or more of the following criteria must be met:  
*** This spread is to:
* [[Unresectable]] primary tumor.
**** [[Lungs]]
* Lymph node [[metastasis]].
**** [[Liver]]
* Positive surgical margins.
**** [[Bone]]
*Preoperative chemotherapy has been given.
**** [[Brain]]
 
*** Lymph nodes can be involved beyond the abdominopelvic region.
* [[Tumor spillage]] involving peritoneal surfaces either before or during surgery, or [[transected]] tumor thrombus.
** '''Stage 5'''
 
*** Both kidneys are involved at the time of the diagnosis.  
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.


==References==
==References==

Latest revision as of 05:27, 10 July 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sargun Singh Walia M.B.B.S.[2]

Overview

Staging of wilms tumor is done based upon the extent of tumor anatomically and not done on the basis of its genetics, histology or molecular markers. Extensive disease and worse prognosis is denoted by a higher stage. The staging systems that are currently used are National Wilms Tumor Study (NWTS) system and International Society of Pediatric Oncology (SIOP) system. Both these systems divide wilms tumor into 5 stages.

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.

NWTS system 

  • This system is used throughout United States and Canada.
  • It is based on the evaluations done during surgery before the administration of surgery.
  • According to this system wilms tumor is divided into following stages.[2]
    • Stage 1
      • Tumor is limited only to the kidney.
      • When the tumor is resected the capsule is intact.
      • Resected tumor shows no evidence of involvement of renal sinus vessel.
    • Stage 2
      • Tumor is extended beyond the kidney, but can be resected completely.
      • Tumor margins go beyond the renal capsule.
      • It extends into the renal capsule, soft tissues and blood vessels beyond the renal parenchyma.
      • But all this is within the margins of the resected tumor.
    • Stage 3
      • Complete tumor cannot be resected.
      • The remaining tumor is confined within the abdomen.
      • Residual tumor remaining within abdomen includes :
    • Stage 4
    • Stage 5
      • When Wilms tumor is diagnosed, bilateral renal involvement is seen.
      • Each kidney has to be staged separately.

SIOP staging

  • This system is followed in Europe.
  • This system is based on the surgical evaluation done after the size of the tumor is reduced after chemotherapy.
  • According to this system wilms tumor is divided into following stages.
    • Stage 1
      • Tumor is limited only to the kidney.
      • If the tumor extends beyond the kidney, a fibrous pseudocapsule surrounds it.
      • Tumor can be found in the pelvic system but should not involve ureter walls.
    • Stage 2
      • Tumor is extended beyond the kidney, but can be resected completely.
      • After resection there is no evidence beyond the resection margins.
    • Stage 3
      • After tumor is resected surgically, some residual tumor still remains.
      • This remainder tumor is confined to the abdomen.
      • No hematogenous metastasis is present.
    • Stage 4
      • Hematogenous spread os positive.
      • This spread is to:
      • Lymph nodes can be involved beyond the abdominopelvic region.
    • Stage 5
      • Both kidneys are involved at the time of the diagnosis.

References

  1. ↑ Fujita H (1989). "Freeze-fracture method and its application to the cell biology". J Electron Microsc (Tokyo). 38 Suppl: S110–7. PMID 2809467.
  2. ↑ Metzger ML, Dome JS (2005). "Current therapy for Wilms' tumor". Oncologist. 10 (10): 815–26. doi:10.1634/theoncologist.10-10-815. PMID 16314292.

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