Results of the Third AIEOP Cooperative Protocol on Wilms Tumor (TW2003) and Related Considerations

Filippo Spreafico, Davide Biasoni, Salvatore Lo Vullo, Lorenza Gandola, Paolo D'Angelo, Monica Terenziani, Maurizio Bianchi, Massimo Provenzi, Paolo Indolfi, Andrea Pession, Marilina Nantron, Andrea Di Cataldo, Carlo Morosi, Daniela Perotti, Serena Catania, Franca Fossati Bellani, Paola Collini, AIEOP Wilms Tumor Working Group

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Abstract

PURPOSE: TW2003, the third Italian prospective study on Wilms tumor, aimed to improve survival in patients with stage III-IV tumors, de-escalate therapy for stage I-II nonanaplastic tumors, refine the risk stratification of therapy, and develop a national infrastructure for biobanking and central pathology review.

MATERIALS AND METHODS: TW2003 recruited children 18 years old or younger with primary intrarenal tumors. Local physicians chose nephrectomy with or without preoperative chemotherapy as the initial treatment based on the risk of unsafe and/or incomplete immediate surgery. The main drivers for adjuvant therapy were tumor stage and diffuse anaplasia. A new risk stratification schema was investigated, incorporating patient age, reason for stage III designation and completeness of lung nodule response in stage IV disease.

RESULTS: We report on 453 patients with unilateral Wilms tumor. Preoperative chemotherapy was administered to 42% of patients. The 5-year event-free survival and overall survival rates were 89.1% (95% CI 83.6-94.9) and 97.0% (93.7-100) for stage I; 85.1% (79.6-91.1) and 94.0% (90.1-98.1) for stage II (160); 82.7% (75.3-90.8) and 90.9% (85.0-97.1) for stage III (101); and 72.1% (61.9-84.0) and 82.5% (73.1-93.1) for stage IV (69), respectively. On multivariable analysis only anaplasia was significant for event-free survival (HR 2.68, 95% CI 1.48-4.86, p=0.001; bias corrected c-index 0.580) and overall survival (HR 5.29, 95% CI 2.52-11.12, p <0.001; bias corrected c-index 0.697).

CONCLUSIONS: The survival rates achieved and the proposed risk stratification schema provide a basis for future comparisons of Wilms tumor treatment burden and patient outcome.

Original languageEnglish
Pages (from-to)1138-1145
Number of pages8
JournalJournal of Urology
Volume198
Issue number5
DOIs
Publication statusPublished - Nov 2017

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Wilms Tumor
Anaplasia
Disease-Free Survival
Neoplasms
Survival Rate
Therapeutics
Drug Therapy
Survival
Tumor Burden
Nephrectomy
Prospective Studies
Pathology
Physicians
Lung

Keywords

  • Journal Article

Cite this

Spreafico, F., Biasoni, D., Lo Vullo, S., Gandola, L., D'Angelo, P., Terenziani, M., ... AIEOP Wilms Tumor Working Group (2017). Results of the Third AIEOP Cooperative Protocol on Wilms Tumor (TW2003) and Related Considerations. Journal of Urology, 198(5), 1138-1145. https://doi.org/10.1016/j.juro.2017.06.081

Results of the Third AIEOP Cooperative Protocol on Wilms Tumor (TW2003) and Related Considerations. / Spreafico, Filippo; Biasoni, Davide; Lo Vullo, Salvatore; Gandola, Lorenza; D'Angelo, Paolo; Terenziani, Monica; Bianchi, Maurizio; Provenzi, Massimo; Indolfi, Paolo; Pession, Andrea; Nantron, Marilina; Di Cataldo, Andrea; Morosi, Carlo; Perotti, Daniela; Catania, Serena; Bellani, Franca Fossati; Collini, Paola; AIEOP Wilms Tumor Working Group.

In: Journal of Urology, Vol. 198, No. 5, 11.2017, p. 1138-1145.

Research output: Contribution to journalArticle

Spreafico, F, Biasoni, D, Lo Vullo, S, Gandola, L, D'Angelo, P, Terenziani, M, Bianchi, M, Provenzi, M, Indolfi, P, Pession, A, Nantron, M, Di Cataldo, A, Morosi, C, Perotti, D, Catania, S, Bellani, FF, Collini, P & AIEOP Wilms Tumor Working Group 2017, 'Results of the Third AIEOP Cooperative Protocol on Wilms Tumor (TW2003) and Related Considerations', Journal of Urology, vol. 198, no. 5, pp. 1138-1145. https://doi.org/10.1016/j.juro.2017.06.081
Spreafico F, Biasoni D, Lo Vullo S, Gandola L, D'Angelo P, Terenziani M et al. Results of the Third AIEOP Cooperative Protocol on Wilms Tumor (TW2003) and Related Considerations. Journal of Urology. 2017 Nov;198(5):1138-1145. https://doi.org/10.1016/j.juro.2017.06.081
Spreafico, Filippo ; Biasoni, Davide ; Lo Vullo, Salvatore ; Gandola, Lorenza ; D'Angelo, Paolo ; Terenziani, Monica ; Bianchi, Maurizio ; Provenzi, Massimo ; Indolfi, Paolo ; Pession, Andrea ; Nantron, Marilina ; Di Cataldo, Andrea ; Morosi, Carlo ; Perotti, Daniela ; Catania, Serena ; Bellani, Franca Fossati ; Collini, Paola ; AIEOP Wilms Tumor Working Group. / Results of the Third AIEOP Cooperative Protocol on Wilms Tumor (TW2003) and Related Considerations. In: Journal of Urology. 2017 ; Vol. 198, No. 5. pp. 1138-1145.
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T1 - Results of the Third AIEOP Cooperative Protocol on Wilms Tumor (TW2003) and Related Considerations

AU - Spreafico, Filippo

AU - Biasoni, Davide

AU - Lo Vullo, Salvatore

AU - Gandola, Lorenza

AU - D'Angelo, Paolo

AU - Terenziani, Monica

AU - Bianchi, Maurizio

AU - Provenzi, Massimo

AU - Indolfi, Paolo

AU - Pession, Andrea

AU - Nantron, Marilina

AU - Di Cataldo, Andrea

AU - Morosi, Carlo

AU - Perotti, Daniela

AU - Catania, Serena

AU - Bellani, Franca Fossati

AU - Collini, Paola

AU - AIEOP Wilms Tumor Working Group

N1 - Copyright © 2017 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

PY - 2017/11

Y1 - 2017/11

N2 - PURPOSE: TW2003, the third Italian prospective study on Wilms tumor, aimed to improve survival in patients with stage III-IV tumors, de-escalate therapy for stage I-II nonanaplastic tumors, refine the risk stratification of therapy, and develop a national infrastructure for biobanking and central pathology review.MATERIALS AND METHODS: TW2003 recruited children 18 years old or younger with primary intrarenal tumors. Local physicians chose nephrectomy with or without preoperative chemotherapy as the initial treatment based on the risk of unsafe and/or incomplete immediate surgery. The main drivers for adjuvant therapy were tumor stage and diffuse anaplasia. A new risk stratification schema was investigated, incorporating patient age, reason for stage III designation and completeness of lung nodule response in stage IV disease.RESULTS: We report on 453 patients with unilateral Wilms tumor. Preoperative chemotherapy was administered to 42% of patients. The 5-year event-free survival and overall survival rates were 89.1% (95% CI 83.6-94.9) and 97.0% (93.7-100) for stage I; 85.1% (79.6-91.1) and 94.0% (90.1-98.1) for stage II (160); 82.7% (75.3-90.8) and 90.9% (85.0-97.1) for stage III (101); and 72.1% (61.9-84.0) and 82.5% (73.1-93.1) for stage IV (69), respectively. On multivariable analysis only anaplasia was significant for event-free survival (HR 2.68, 95% CI 1.48-4.86, p=0.001; bias corrected c-index 0.580) and overall survival (HR 5.29, 95% CI 2.52-11.12, p <0.001; bias corrected c-index 0.697).CONCLUSIONS: The survival rates achieved and the proposed risk stratification schema provide a basis for future comparisons of Wilms tumor treatment burden and patient outcome.

AB - PURPOSE: TW2003, the third Italian prospective study on Wilms tumor, aimed to improve survival in patients with stage III-IV tumors, de-escalate therapy for stage I-II nonanaplastic tumors, refine the risk stratification of therapy, and develop a national infrastructure for biobanking and central pathology review.MATERIALS AND METHODS: TW2003 recruited children 18 years old or younger with primary intrarenal tumors. Local physicians chose nephrectomy with or without preoperative chemotherapy as the initial treatment based on the risk of unsafe and/or incomplete immediate surgery. The main drivers for adjuvant therapy were tumor stage and diffuse anaplasia. A new risk stratification schema was investigated, incorporating patient age, reason for stage III designation and completeness of lung nodule response in stage IV disease.RESULTS: We report on 453 patients with unilateral Wilms tumor. Preoperative chemotherapy was administered to 42% of patients. The 5-year event-free survival and overall survival rates were 89.1% (95% CI 83.6-94.9) and 97.0% (93.7-100) for stage I; 85.1% (79.6-91.1) and 94.0% (90.1-98.1) for stage II (160); 82.7% (75.3-90.8) and 90.9% (85.0-97.1) for stage III (101); and 72.1% (61.9-84.0) and 82.5% (73.1-93.1) for stage IV (69), respectively. On multivariable analysis only anaplasia was significant for event-free survival (HR 2.68, 95% CI 1.48-4.86, p=0.001; bias corrected c-index 0.580) and overall survival (HR 5.29, 95% CI 2.52-11.12, p <0.001; bias corrected c-index 0.697).CONCLUSIONS: The survival rates achieved and the proposed risk stratification schema provide a basis for future comparisons of Wilms tumor treatment burden and patient outcome.

KW - Journal Article

U2 - 10.1016/j.juro.2017.06.081

DO - 10.1016/j.juro.2017.06.081

M3 - Article

C2 - 28655531

VL - 198

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JO - Journal of Urology

JF - Journal of Urology

SN - 0022-5347

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