Omission of adjuvant chemotherapy in patients with completely necrotic Wilms tumor stage I and radiotherapy in stage III: The 30-year SIOP-RTSG experience.
Wilms tumor
completely necrotic type
radiotherapy
reduced treatment
Journal
Pediatric blood & cancer
ISSN: 1545-5017
Titre abrégé: Pediatr Blood Cancer
Pays: United States
ID NLM: 101186624
Informations de publication
Date de publication:
07 Jan 2024
07 Jan 2024
Historique:
revised:
27
11
2023
received:
19
09
2023
accepted:
21
12
2023
medline:
8
1
2024
pubmed:
8
1
2024
entrez:
7
1
2024
Statut:
aheadofprint
Résumé
Completely necrotic Wilms tumor (CN-WT) following preoperative chemotherapy has been regarded as low-risk WT since the International Society of Paediatric Oncology (SIOP) 93-01 study, and patients have been treated with reduced postoperative therapy. The aim of the study was to evaluate whether the omission of adjuvant chemotherapy in patients with localized CN-WT stage I and radiotherapy in stage III was safe. The retrospective observational study of outcomes of patients diagnosed with localized CN-WT on central pathology review and treated according to the SIOP 93-01 and SIOP-WT-2001 protocols (1993-2022). There were 125 patients with localized CN-WT: 90 with stage I, 10 with stage II, and 25 with stage III. Sixty-two of 125 (49.6%) patients had a discrepant diagnosis and/or staging between the institutional pathologist and central pathology review. In the group of 90 patients with stage I, postoperative chemotherapy was not given to 41 (46%) patients, whereas 49 patients received postoperative chemotherapy-in the latter group, two patients relapsed, and one of them died. One stage I and one stage II patient developed chemotherapy-induced toxicity and died. Nineteen of 25 patients with stage III received no flank radiotherapy. No stage III patient relapsed or died. The overall 5-year event-free survival (EFS) estimate for the entire cohort (stages I-III) was 96.8% [95% confidence interval, CI: 93.6%-99.6%] and the overall survival (OS) was 97.6% [95% CI: 95.0-100%]. The EFS and OS were 97% and 98%, respectively, for stage I, and 100% for stage III. Omission of postoperative chemotherapy for patients with CN-WT stage I, and radiotherapy for stage III is safe. Rapid central pathology review is required to assign appropriate treatment and avoid treatment-related side effects.
Sections du résumé
BACKGROUND
BACKGROUND
Completely necrotic Wilms tumor (CN-WT) following preoperative chemotherapy has been regarded as low-risk WT since the International Society of Paediatric Oncology (SIOP) 93-01 study, and patients have been treated with reduced postoperative therapy. The aim of the study was to evaluate whether the omission of adjuvant chemotherapy in patients with localized CN-WT stage I and radiotherapy in stage III was safe.
PATIENTS AND METHODS
METHODS
The retrospective observational study of outcomes of patients diagnosed with localized CN-WT on central pathology review and treated according to the SIOP 93-01 and SIOP-WT-2001 protocols (1993-2022).
RESULTS
RESULTS
There were 125 patients with localized CN-WT: 90 with stage I, 10 with stage II, and 25 with stage III. Sixty-two of 125 (49.6%) patients had a discrepant diagnosis and/or staging between the institutional pathologist and central pathology review. In the group of 90 patients with stage I, postoperative chemotherapy was not given to 41 (46%) patients, whereas 49 patients received postoperative chemotherapy-in the latter group, two patients relapsed, and one of them died. One stage I and one stage II patient developed chemotherapy-induced toxicity and died. Nineteen of 25 patients with stage III received no flank radiotherapy. No stage III patient relapsed or died. The overall 5-year event-free survival (EFS) estimate for the entire cohort (stages I-III) was 96.8% [95% confidence interval, CI: 93.6%-99.6%] and the overall survival (OS) was 97.6% [95% CI: 95.0-100%]. The EFS and OS were 97% and 98%, respectively, for stage I, and 100% for stage III.
CONCLUSION
CONCLUSIONS
Omission of postoperative chemotherapy for patients with CN-WT stage I, and radiotherapy for stage III is safe. Rapid central pathology review is required to assign appropriate treatment and avoid treatment-related side effects.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e30852Informations de copyright
© 2024 Wiley Periodicals LLC.
Références
Graf N, Bergeron C, Brok J, et al. Fifty years of clinical and research studies for childhood renal tumors within the International Society of Pediatric Oncology (SIOP). Ann Oncol. 2021;32:1327-1331.
Vujanić GM, Sandstedt B, Kelsey A, Sebire NJ. Central pathology review in multicenter trials and studies: lessons from the nephroblastoma trials. Cancer. 2009;115:1977-1983.
Vujanić GM, Sandstedt B. The pathology of Wilms' tumor (nephroblastoma): the International Society of Paediatric Oncology approach. J Clin Pathol. 2010;63:102-109.
Boccon-Gibod L, Rey A, Sandstedt B, et al. Complete necrosis induced by preoperative chemotherapy in Wilms tumor as an indicator of low risk: report of the International Society of Paediatric Oncology (SIOP) nephroblastoma trial and study 9. Med Pediatr Oncol. 2000;34:183-190.
Delemarre JF, Sandstedt B, Harms D, Boccon-Gibod L, Vujanić GM. The new SIOP (Stockholm) working classification of renal tumors of childhood. Med Pediatr Oncol. 1996;26:145-146.
Vujanić GM, Sandstedt B, Harms D, Kelsey A, Leuschner I, de Kraker J. Revised International Society of Paediatric Oncology (SIOP) working classification of renal tumors of childhood. Med Pediatr Oncol. 2002;38:79-82.
Vujanić GM, Gessler M, Ooms AHAG, et al. The Umbrella SIOP-RTSG 2016 Study Wilms tumour pathology and molecular biology protocol. Nat Rev Urol. 2018;15:693-701.
de Kraker J, Graf N, van Tinteren H, et al. Reduction of postoperative chemotherapy in children with stage I intermediate-risk and anaplastic Wilms’ tumor (SIOP 93-01 trial): a randomised controlled trial. Lancet. 2004;364:1229-1235.
Pritchard-Jones K, Bergeron C, de Camargo B, et al. Omission of doxorubicin from the treatment of stage II-III, intermediate-risk Wilms' tumor (SIOP WT 2001): an open-label, non-inferiority, randomised controlled trial. Lancet. 2015;386:1156-1164.
R Core Team. R: a language and environment for statistical computing. R Foundation for Statistical Computing; 2018. Accessed 6 June 2023. https://www.R-project.org
Tournade MF, Com-Nougue C, De Kraker J, et al. Optimal duration of preoperative therapy in unilateral and nonmetastatic Wilms’ tumor in children older than 6 months: results of the Ninth International Society of Pediatric Oncology Wilms’ Tumor Trial and Study. J Clin Oncol. 2001;19:488-500.
Vujanić GM, D'Hooghe E, Graf N, et al. Prognostic significance of histopathological response to preoperative chemotherapy in unilateral Wilms tumor: an analysis of 899 patients treated on the SIOP WT 2001 protocol in the UK CCLG and GPOH studies. Int J Cancer. 2021;149:1332-1340.
Weirich A, Leuschner I, Harms D, et al. Clinical impact of histologic subtypes in localized non-anaplastic nephroblastoma treated according to the trial and study SIOP-9/GPOH. Ann Oncol. 2001;12:311-319.
Vujanić GM, Apps JR, Moroz V, et al. Nephrogenic rests in Wilms tumors treated with preoperative chemotherapy: the UK SIOP Wilms Tumor 2001 Trial experience. Pediatr Blood Cancer. 2017;64:e26547.
Nakata K, Williams R, Kinoshita Y, et al. Comparative analysis of the clinical characteristics and outcomes of patients with Wilms tumor in the United Kingdom and Japan. Pediatr Blood Cancer. 2012;68:e29143.
Davila Fajardo R, Furtwängler R, van Grotel M, et al. Outcome of stage IV completely necrotic Wilms tumour and local stage III treated according to the SIOP 2001 protocol. Cancers. 2021;13:976.
de Aguirre-Neto JC, de Camargo B, van Tinteren H, et al. International comparisons of clinical demographics and outcomes in the International Society of Pediatric Oncology Wilms Tumor 2001 Trial and Study. JCO Glob Oncol. 2022;8:e2100425.
Bisogno G, de Kraker J, Weirich A, et al. Veno-occlusive disease of the liver in children treated for Wilms tumor. Med Pediatr Oncol. 1997;29:245-251.
Jagt CT, Zuckermann M, Ten Kate F, et al. Veno-occlusive disease as a complication of preoperative chemotherapy for Wilms tumor: a clinico-pathological analysis. Pediatr Blood Cancer. 2009;53:1211-1215.
Beijer JGM, Kok JL, Janssens GO, et al. Adverse late health outcomes among children treated with 3D radiotherapy techniques: study design of the Dutch pediatric 3D-RT study. Cancer Rep. 2023;6:e1620.
Mohty M, Battista ML, Blaise D, et al. A multicentre, multinational, prospective, observational registry study of defibrotide in patients diagnosed with veno-occlusive disease/sinusoidal obstruction syndrome after haematopoietic cell transplantation: an EBMT study. Bone Marrow Transplant. 2021;56:2454-2463.