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Wilms Tumor



Very High Risk Renal Tumors

The COG research study for very high risk renal tumors was AREN0321. The standard of care adopted by most institutions in North America would be to treat children with very high risk renal tumors according to this studies' protocol guidelines until another study is available

The pathology types below make up a very small proportion of renal tumors in children, but account for most deaths.


Very high risk renal tumors eligible for AREN0321:


Proportion of all Renal Tumors

Anaplastic Wilms tumor


Clear cell sarcoma of the kidney (CCSK)


Malignant rhabdoid tumor (MRT)


Renal cell carcinoma (RCC)



Anaplastic Wilms Tumor

  • Anaplastic histology is the single most important histologic predictor of response and survival in patients with Wilms tumor.
  • Focal anaplasia does not confer as poor a prognosis as does diffuse anaplasia.
  • Stage I diffuse anaplastic histology did not do well in NWTS-5 and so their treatment is intensified (see table below).


  • NWTS-3 and NWTS-4 treated diffuse anaplastic Wilms tumor patients with 15 months of vincristine, actinomycin D, and doxorubicin. Also patients were randomized to receive additional cyclophosphamide.
  • Patients with Stages II-IV diffuse anaplastic histology had a 4-year relapse-free survival (RFS) of about 27% if they did NOT get cyclophosphamide vs 55% with addition of cyclophosphamide.
  • NWTS-5 included cyclophosphamide into treatment protocol for these patients.


  • Most of these tumors have TP53 mutations.
  • TP53 mutations are very uncommon in favorable histology.
  • Microdissection analysis of tumors showed that TP53 mutations are restricted to areas of anaplasia.
  • p53 induces cell cycle arrest or apoptosis in response to DNA damage - so inactivation of p53 may promote resistance to anti-cancer therapy.


Chemotherapy Treatment Regimes:

Tumor and Stage Treatment Regimen

Focal anaplastic WT

  • Stage I-III

Diffuse anaplastic WT

  • Stage I

Regimen DD-4A


(vincristine/dactinomycin/doxorubicin x 25 weeks + Flank RT)


Focal anaplastic WT

  • Stage IV

Diffuse anaplastic WT

  • Stage II-III

Diffuse anaplastic WT

  • Stage IV
  • without measurable disease

Malignant Rhabdoid Tumor

  • Stage I-III
  • without measurable disease

Clear Cell Sarcoma Kidney

  • Stage IV

Regimen UH1


(cyclophosphamide/carboplatin/etoposide; vincristine/doxorubicin/cyclophosphamide; x 30 weeks + RT)

Clear Cell Sarcoma Kidney

  • Stage I-III

Regimen I

(vincristine/doxorubicin/cyclophosphamide; cyclophosphamide/etoposide x25 weeks; RT)


Renal Cell Carcinoma

  • Stage I-IV
  • completely resected disease
Surgery only

Renal Cell Carcinoma

  • Stage IV
Institutional Preference

Diffuse anaplastic WT

  • Stage IV
  • with measurable disease

Malignant Rhabdoid Tumor

  • Stage III –IV
  • with measurable disease
Window Therapy#


Indications for Flank or whole abdominal RT:

Wilms Tumor: Focal Anaplasia Abdominal stage I, II, III

Wilms Tumor: Diffuse Anaplasia

Abdominal stage I, II, III

Clear Cell Sarcoma

Abdominal stage II, III

Stage I only if node sampling and / or pathology review not done

Rhabdoid Tumor Abdominal stage I, II, III



Indications & RT Dose

Flank RT

  • Stage I-III Focal Anaplasia - 10.8 Gy
  • Stage I,II Diffuse Anaplasia - 10.8 Gy
  • Stage III Diffuse Anaplasia - 19.8 Gy
  • Stage I-III MRT - 19.8 Gy for patients more than 1y & 10.8 Gy for patients less than 1y
  • Stage I CCSK - 0-10.8 Gy
  • Stage II,III CCSK - 10.8 Gy
  • Stage V-1080 cGy – 19.8 Gy


Whole Abdomen RT

Stage III with:

  • Cytology-positive ascites
  • Pre-operative intraperitoneal rupture
  • Diffuse operative spill
  • Peritoneal seeding


Doses used as above for flank RT but at 150 cGy per fraction.


When dose to whole abdomen is more than 10.5 Gy (diffuse anaplasia or MRT), renal shielding limits dose to the normal kidney to less than 14.4 Gy

Whole Lung RT

  • Lung metastases - 12 Gy at 150 cGy per fraction; 10.5 Gy for patients less than 12 months old


Lymph Node RT

  • Lymph node metastasis not surgically removed - 19.8 Gy in 180 cGy #


Liver RT

  • Liver metastases - 19.8 Gy


Bone RT
  • Bone metastases - 25.2 Gy


A boost of 10.8 Gy is given to residual tumor after surgery



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