Purpose: Treatment options for stage I favourable histology WT range over protocols from nephrectomy-only to adjuvant 19 weeks of vincristine-actinomycin D (VA).1,2 We reviewed the results concerning the children with stage IWT treated according to the AIEOP-TW-2003 protocol. Methods: The AIEOP-TW-2003 approaches for stage I tumours included: adjuvant 6-week VA (13 children received further 4 weeks of VA preoperatively); nephrectomy alone for children younger than 2 years with small (<550 g), epithelial-type tumours. Results: Ninety-six stage I WTs (7 had diffuse anaplasia) of 357 eligible monolateral WTs were the object of this analysis. Median aged was 21 months (range 1–89; 53 children aged 2 years). Five children entered the nephrectomy-only trial. At 55 months (median) of follow-up the 4-year EFS and OS rates were 88.1% ( 3.4%) and 97.7% (1.6%), respectively, for the series as a whole, and 89.7% (3.3%) and 97.6% (1.7%) for the 91 patients who received adjuvant chemotherapy. There were 10 relapses (contralateral kidney, 4–with lung in one case; distant, 5; local, 1); one child eventually died. Two of the 10 tumours that relapsed were centrally reclassified as stage II, one had diffuse anaplasia, and two were initially treated by surgery alone. One A-related toxic death occurred. Lymph nodes were not sampled in 26 children. The age >2 years (p ¼ .6) and failure to sample lymphnodes (p ¼ .3) did not seem to influence outcome. Conclusion: The outcome for stage IWT is excellent. The short 6-week VA regimen did not seem to prevent the evolution of nephrogenic rests into tumours (40% of relapses were metachronous WTs) nor to significantly improve the EFS comparing to a surgery-only strategy like the COG one for young children. A finer tuning of the therapy for stage I WT should be driven by clinical, histological and genetic parameters. References [1] Frazier AL, Shamberger RC, Henderson TO, Diller L. Ped Blood Cancer 2010;54:879; [2] Pritchard-Jones K. Ped Blood Cancer 2010;54:865.

LOOKING FOR THE OPTIMAL THERAPY FOR STAGE I WILMS TUMOUR (WT). DATA FROM THE ASSOCIAZIONE ITALIANA EMATOLOGIA ONCOLOGIA PEDIATRICA (AIEOP) TW-2003 PROTOCOL

DI CATALDO, Andrea;
2012-01-01

Abstract

Purpose: Treatment options for stage I favourable histology WT range over protocols from nephrectomy-only to adjuvant 19 weeks of vincristine-actinomycin D (VA).1,2 We reviewed the results concerning the children with stage IWT treated according to the AIEOP-TW-2003 protocol. Methods: The AIEOP-TW-2003 approaches for stage I tumours included: adjuvant 6-week VA (13 children received further 4 weeks of VA preoperatively); nephrectomy alone for children younger than 2 years with small (<550 g), epithelial-type tumours. Results: Ninety-six stage I WTs (7 had diffuse anaplasia) of 357 eligible monolateral WTs were the object of this analysis. Median aged was 21 months (range 1–89; 53 children aged 2 years). Five children entered the nephrectomy-only trial. At 55 months (median) of follow-up the 4-year EFS and OS rates were 88.1% ( 3.4%) and 97.7% (1.6%), respectively, for the series as a whole, and 89.7% (3.3%) and 97.6% (1.7%) for the 91 patients who received adjuvant chemotherapy. There were 10 relapses (contralateral kidney, 4–with lung in one case; distant, 5; local, 1); one child eventually died. Two of the 10 tumours that relapsed were centrally reclassified as stage II, one had diffuse anaplasia, and two were initially treated by surgery alone. One A-related toxic death occurred. Lymph nodes were not sampled in 26 children. The age >2 years (p ¼ .6) and failure to sample lymphnodes (p ¼ .3) did not seem to influence outcome. Conclusion: The outcome for stage IWT is excellent. The short 6-week VA regimen did not seem to prevent the evolution of nephrogenic rests into tumours (40% of relapses were metachronous WTs) nor to significantly improve the EFS comparing to a surgery-only strategy like the COG one for young children. A finer tuning of the therapy for stage I WT should be driven by clinical, histological and genetic parameters. References [1] Frazier AL, Shamberger RC, Henderson TO, Diller L. Ped Blood Cancer 2010;54:879; [2] Pritchard-Jones K. Ped Blood Cancer 2010;54:865.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11769/111648
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