Wilms tumor

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Last updated on 2024-07-23:3 regimens on this page
3 variants on this page
=Guidelines=
Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.
==NCCN==
*NCCN Guidelines - Wilms Tumor (Nephroblastoma)
=Neoadjuvant therapy=
==Vincristine monotherapy==
===Regimen===
Study 	Dates of enrollment	Evidence
Sullivan et al. 1967	1965-1966 	Pilot
====Chemotherapy====
*Vincristine (Oncovin) 0.05 mg/kg IV
One or two doses
====Subsequent treatment====
*Surgical resection
===References===
# 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 dosing details in manuscript have been reviewed by our editors EuroPMC
# 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. link to original article EuroPMC
=Adjuvant therapy=
==Dactinomycin monotherapy==
===Regimen===
Study 	Dates of enrollment 	Evidence 	Comparator 	Comparative Efficacy
Wolff et al. 1968 	1964 to not reported	Randomized (E-esc)	Dactinomycin x 1 	Seems to have longer RFS
D'Angio et al. 1976 (NWTS-1)	1969-1973 	Randomized (C) 	1. Dactinomycin & Vincristine	Shorter RFS
D'Angio et al. 1976 (NWTS-1)	1969-1973 	Randomized (C) 	2. Vincristine 	Not reported
====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===
# 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 EuroPMC
## 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 dosing details in manuscript have been reviewed by our editors EuroPMC
# 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. link to original article EuroPMC
==Dactinomycin & Vincristine==
===Regimen===
Study 	Dates of enrollment	Evidence 	Comparator 	Comparative Efficacy
D'Angio et al. 1976 (NWTS-1) 	1969-1973 	Randomized (E-RT-esc) 	1. Dactinomycin2. Vincristine 	Longer RFS
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
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 EFS24 (primary endpoint)
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) 45 mcg/kg (maximum dose of 2 mg) IV bolus once on day 8
*Vincristine (Oncovin) as follows:
**Cycles 1 & 2: 1.5 mg/m2 (maximum dose of 2 mg) IV bolus once per day on days 1, 8, 15
**Cycle 3: 1.5 mg/m2 (maximum dose of 2 mg) IV bolus once per day on days 1 & 8
**Cycles 4 to 9: 1.5 mg/m2 (maximum dose of 2 mg) IV bolus once per day on days 8 & 15
21-day cycle for 9 cycles
===References===
# 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. link to original article EuroPMC
# 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. link to original article EuroPMC
# 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. link to original article EuroPMC
# 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 EuroPMC
# 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 EuroPMC NCT00003804
# 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 dosing details in manuscript have been reviewed by our editors EuroPMC NCT00047138
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