Wilms tumor

Section editor transclusions

3 regimens on this page 3 variants on this page

Guidelines

NCCN

• NCCN Guidelines - Wilms Tumor (Nephroblastoma)

All lines of therapy

Dactinomycin monotherapy

Regimen

Study	Years of enrollment	Evidence	Comparator	Comparative Efficacy
Wolff et al. 1968	1964-NR	Randomized (E-esc)	Dactinomycin x 1	Seems to have superior RFS

Preceding treatment

• Surgical resection

Chemotherapy

• Dactinomycin (Cosmegen) 15 mcg/kg IV once per day on days 1 to 5

6-week cycle for 3 cycles, then 3-month cycle for 4 cycles

References

1. Wolff JA, Newton WA Jr, Krivit W, D'Angio GJ. Single versus multiple dose dactinomycin therapy of Wilms's tumor: a controlled co-operative study conducted by the Children's Cancer Study Group A (formerly Acute Leukemia Co-operative Chemotherapy Group A). N Engl J Med. 1968 Aug 8;279(6):290-4. link to original article PubMed
   1. Update: Wolff JA, D'Angio G, Hartmann J, Krivit W, Newton WA Jr. Long-term evaluation of single versus multiple courses of actinomycin D therapy of Wilms's tumor. N Engl J Med. 1974 Jan 10;290(2):84-6. link to original article contains dosing details in manuscript PubMed

Dactinomycin & Vincristine

Regimen

Study	Years of enrollment	Evidence	Comparator	Comparative Efficacy
<633::AID-CNCR2820380203%3E3.0.CO;2-S D'Angio et al. 1976 (NWTS-1)	1969-1973	Randomized (E-RT-esc)	1. Dactinomycin 2. Vincristine	Superior RFS
<2302::AID-CNCR2820470933%3E3.0.CO;2-K D'Angio et al. 1981 (NWTS-2)	1974-1978	Randomized (E-RT-de-esc)	Dactinomycin & Vincristine; longer duration	Did not meet primary endpoint of RFS
<349::AID-CNCR2820640202%3E3.0.CO;2-Q D'Angio et al. 1989 (NWTS-3)	1979-1985	Randomized (E-RT-de-esc)	Dactinomycin & Vincristine; longer duration	Did not meet primary endpoint of RFS
Ritchey et al. 2001 (NWTS-4)	1986-1994	Non-randomized (RT)
de Kraker et al. 2004 (SIOP 93-01)	1993-2000	Phase 3 (E-de-esc)	Prolonged chemotherapy	Equivalent EFS
Pritchard-Jones et al. 2015 (SIOP WT 2001)	2001-2009	Phase 3 (E-de-esc)	Dactinomycin, Doxorubicin, Vincristine	Non-inferior EFS

Note: Ritchey et al. 2001 does not appear to be a primary publication of the NWTS-4 results, but no other publications could be found; details including phase of study are unclear.

Preceding treatment

• Surgical resection

Chemotherapy

• Dactinomycin (Cosmegen)
• Vincristine (Oncovin)

References

1. NWTS-1: D'Angio GJ, Evans AE, Breslow N, Beckwith B, Bishop H, Feigl P, Goodwin W, Leape LL, Sinks LF, Sutow W, Tefft M, Wolff J. The treatment of Wilms' tumor: Results of the national Wilms' tumor study. Cancer. 1976 Aug;38(2):633-46. <633::AID-CNCR2820380203%3E3.0.CO;2-S link to original article PubMed
2. NWTS-2: D'Angio GJ, Evans A, Breslow N, Beckwith B, Bishop H, Farewell V, Goodwin W, Leape L, Palmer N, Sinks L, Sutow W, Tefft M, Wolff J. The treatment of Wilms' tumor: results of the Second National Wilms' Tumor Study. Cancer. 1981 May 1;47(9):2302-11. <2302::AID-CNCR2820470933%3E3.0.CO;2-K link to original article PubMed
3. NWTS-3: D'Angio GJ, Breslow N, Beckwith JB, Evans A, Baum H, deLorimier A, Fernbach D, Hrabovsky E, Jones B, Kelalis P, Othersen B, Tefft M, Thomas PRM. Treatment of Wilms' tumor: results of the Third National Wilms' Tumor Study. Cancer. 1989 Jul 15;64(2):349-60. <349::AID-CNCR2820640202%3E3.0.CO;2-Q link to original article PubMed
4. NWTS-4: Ritchey ML, Shamberger RC, Haase G, Horwitz J, Bergemann T, Breslow NE. Surgical complications after primary nephrectomy for Wilms' tumor: report from the National Wilms' Tumor Study Group. J Am Coll Surg. 2001 Jan;192(1):63-8. link to original article PubMed
5. SIOP 93-01: de Kraker J, Graf N, van Tinteren H, Pein F, Sandstedt B, Godzinski J, Tournade MF; SIOP. Reduction of postoperative chemotherapy in children with stage I intermediate-risk and anaplastic Wilms' tumour (SIOP 93-01 trial): a randomised controlled trial. Lancet. 2004 Oct 2-8;364(9441):1229-35. link to original article PubMed NCT00003804
6. SIOP WT 2001: Pritchard-Jones K, Bergeron C, de Camargo B, van den Heuvel-Eibrink MM, Acha T, Godzinski J, Oldenburger F, Boccon-Gibod L, Leuschner I, Vujanic G, Sandstedt B, de Kraker J, van Tinteren H, Graf N; SIOP Renal Tumours Study Group. 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 Sep 19;386(9999):1156-64. Epub 2015 Jul 9. link to original article PubMed NCT00047138

Vincristine monotherapy

Regimen

Study	Evidence
Sullivan et al. 1967	Pilot

Chemotherapy

• Vincristine (Oncovin)

Subsequent treatment

• Surgical resection

References

1. Sullivan MP, Sutow WW, Cangir A, Taylor G. Vincristine sulfate in management of Wilms' tumor: replacement of preoperative irradiation by chemotherapy. JAMA. 1967 Oct 30;202(5):381-4. link to original article PubMed