Wilms tumor

Section editor
	Wayne H. Liang, MD, MS UAB Birmingham, AL LinkedIn WayneLiangMD

3 regimens on this page 3 variants on this page

All lines of therapy

Dactinomycin monotherapy

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Regimen

Study	Evidence	Comparator	Efficacy
Wolff et al. 1968	Randomized (E)	Single course of dactinomycin	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 verified protocol PubMed

Dactinomycin & Vincristine

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Regimen

Study	Evidence	Comparator	Efficacy
de Kraker et al. 2004 (SIOP 93-01)	Phase III (E)	Prolonged chemotherapy	Equivalent EFS
Pritchard-Jones et al. 2015 (SIOP WT 2001)	Phase III (E)	Dactinomycin, Doxorubicin, Vincristine	Non-inferior EFS

Preceding treatment

• Surgical resection

Chemotherapy

• Dactinomycin (Cosmegen)
• Vincristine (Oncovin)

References

1. 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
2. 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

Vincristine monotherapy

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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