Treatment regimens for Wilms tumor according to European* (SIOP) protocols

SIOP WT 2001 and 93-01
Stage^¶	Preoperative chemotherapy	Histology^Δ (assessed after initial chemotherapy)	Postoperative chemotherapy	Radiation therapy
I	Vincristine and dactinomycin for 4 weeks	Low risk	None	None
		Intermediate risk	Vincristine and dactinomycin for 4 weeks	None
		High risk	Vincristine, dactinomycin, and doxorubicin for 27 weeks	None
II	Vincristine and dactinomycin for 4 weeks	Low and intermediate risk	Vincristine and dactinomycin for 27 weeks	None
II	Vincristine and dactinomycin for 4 weeks	High risk	Doxorubicin, cyclophosphamide, carboplatin, and etoposide for 34 weeks	25.2 Gy flank; 10.8 Gy boost for lymph node involvement or gross disease
III	Vincristine and dactinomycin for 4 weeks	Low risk	Vincristine and dactinomycin for 27 weeks	None
		Intermediate risk	Vincristine and dactinomycin for 27 weeks	14.4 Gy flank; 10.8 Gy boost for lymph node involvement or gross disease
		High risk	Doxorubicin, cyclophosphamide, carboplatin, and etoposide for 34 weeks	25.2 Gy flank; 10.8 Gy boost for lymph node involvement or gross disease
IV	Vincristine, dactinomycin, and doxorubicin for 6 weeks	Low and intermediate risk; lung nodule with complete response after initial chemotherapy	Vincristine, dactinomycin, and doxorubicin for 27 weeks	No lung XRT; flank XRT for local stage III^◊
		Low and intermediate risk; lung nodule with incomplete response after initial chemotherapy	Doxorubicin, cyclophosphamide, carboplatin, and etoposide for 34 weeks	15 Gy lung; flank XRT for local stage III^◊
		High risk	Doxorubicin, cyclophosphamide, carboplatin, and etoposide for 34 weeks	15 Gy lung; flank XRT for local stage III^◊^§

SIOP: International Society of Paediatric Oncology; XRT: radiation therapy; COG: Children's Oncology Group.
* The SIOP group primarily includes European pediatric oncology centers, with additional centers located in South America and Australia.
¶ Note that the staging systems in SIOP and COG protocols are not equivalent. SIOP stage is determined after preoperative chemotherapy and surgical resection; COG stage is determined before surgical resection.
Δ According to SIOP protocols, Wilms tumors are classified based upon histology as follows: low risk (completely necrotic), intermediate risk (regressive type, mixed type, epithelial type, stromal type, and focal anaplasia), and high risk (blastemal type and diffuse anaplasia).
◊ Metastatic sites other than lung were also irradiated; XRT dose varied according to metastatic site.
§ Lung XRT is not required for patients with blastemal type histology who have complete response (ie, resolution of the lung nodule) with initial chemotherapy.

Additional references:

Pritchard-Jones K, Bergeron C, de Camargo B, et al. Omission of doxorubicin from the treatment of stage II-III, intermediate-risk Wilms' tumour (SIOP WT 2001): An open-label, non-inferiority, randomised controlled trial. Lancet 2015.
Verschuur A, Van Tinteren H, Graf N, et al. Treatment of pulmonary metastases in children with stage IV nephroblastoma with risk-based use of pulmonary radiotherapy. J Clin Oncol 2012; 30:3533.

Adapted from: Dome JS, Graf N, Geller JI, et al. Advances in Wilms tumor treatment and biology: Progress through international collaboration. J Clin Oncol 2015; 33:2999.

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Treatment regimens for Wilms tumor according to European* (SIOP) protocols