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Prognostic significance of age in 5631 patients with Wilms tumour prospectively registered in International Society of Paediatric Oncology (SIOP) 93-01 and 2001.

ABSTRACT: BACKGROUND:To enhance risk stratification for Wilms tumour (WT) in a pre-operative chemotherapy setting, we explored the prognostic significance and optimal age cutoffs in patients treated according to International Society of Paediatric Oncology Renal Tumour Study Group (SIOP-RTSG) protocols. METHODS:Patients(6 months-18 years) with unilateral WT were selected from prospective SIOP 93-01 and 2001 studies(1993-2016). Martingale residual analysis was used to explore optimal age cutoffs. Outcome according to age was analyzed by uni- and multivariable analysis, adjusted for sex, biopsy(yes/no), stage, histology and tumour volume at surgery. RESULTS:5631 patients were included; median age was 3.4 years(IQR: 2-5.1). Estimated 5-year event-free survival (EFS) and overall survival (OS) were 85%(95%CI 83.5-85.5) and 93%(95%CI 92.0-93.4). Martingale residual plots detected no optimal age cutoffs. Multivariable analysis showed lower EFS with increasing age(linear trend P<0.001). Using previously described age categories, EFS was lower for patients aged 2-4(HR 1.34, P = 0.02), 4-10(HR 1.83, P<0.0001) and 10-18 years(HR 1.74, P = 0.01) as compared to patients aged 6 months-2 years. OS was lower for patients 4-10 years(HR 1.67, P = 0.01) and 10-18 years(HR 1.87, P = 0.04), but not for 2-4 years(HR 1.29, P = 0.23). Higher stage, histological risk group and tumour volume were independent adverse prognostic factors. CONCLUSION:Although optimal age cutoffs could not be identified, we demonstrated the prognostic significance of age as well as previously described cutoffs for EFS (2 and 4 years) and OS (4 years) in children with WT treated with pre-operative chemotherapy. These findings encourage the consideration of age in the design of future SIOP-RTSG protocols.

SUBMITTER: Hol JA

PROVIDER: S-EPMC6699693 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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Prognostic significance of age in 5631 patients with Wilms tumour prospectively registered in International Society of Paediatric Oncology (SIOP) 93-01 and 2001.

Hol J A JA Lopez-Yurda M I MI Van Tinteren H H Van Grotel M M Godzinski J J Vujanic G G Oldenburger F F De Camargo B B Ramírez-Villar G L GL Bergeron C C Pritchard-Jones K K Graf N N Van den Heuvel-Eibrink M M MM

PloS one 20190819 8

<h4>Background</h4>To enhance risk stratification for Wilms tumour (WT) in a pre-operative chemotherapy setting, we explored the prognostic significance and optimal age cutoffs in patients treated according to International Society of Paediatric Oncology Renal Tumour Study Group (SIOP-RTSG) protocols.<h4>Methods</h4>Patients(6 months-18 years) with unilateral WT were selected from prospective SIOP 93-01 and 2001 studies(1993-2016). Martingale residual analysis was used to explore optimal age cut ...[more]

PMID: 31425556

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Project description:Wilms tumour (WT) patients with a localised completely necrotic nephroblastoma after preoperative chemotherapy are a favourable outcome group. Since the introduction of the SIOP 2001 protocol, the SIOP- Renal Tumour Study Group (SIOP-RTSG) has omitted radiotherapy for such patients with low-risk, local stage III in an attempt to reduce treatment burden. However, for metastatic patients with local stage III, completely necrotic WT, the recommendations led to ambiguous use. The purpose of this descriptive study is to demonstrate the outcomes of patients with metastatic, completely necrotic and local stage III WT in relation to the application of radiotherapy or not. all metastatic patients with local stage III, completely necrotic WT after 6 weeks of preoperative chemotherapy who were registered in the SIOP 2001 study were included in this analysis. The pattern of recurrence according to the usage of radiation treatment and 5 year event-free survival (EFS) and overall survival (OS) was analysed. seven hundred and three metastatic WT patients were registered in the SIOP 2001 database. Of them, 47 patients had a completely necrotic, local stage III WT: 45 lung metastases (11 combined localisations), 1 liver/peritoneal, and 1 tumour thrombus in the renal vein and the inferior vena cava with bilateral pulmonary arterial embolism. Abdominal radiotherapy was administered in 29 patients (62%; 29 flank/abdominal irradiation and 9 combined with lung irradiation). Eighteen patients did not receive radiotherapy. Median follow-up was 6.6 years (range 1-151 months). Two of the 47 patients (4%) developed disease recurrence in the lung (one combined with abdominal relapse) and eventually died of the disease. Both patients had received abdominal radiotherapy, one of them combined with lung irradiation. Five-year EFS and OS were 95% and 95%, respectively. the outcome of patients with stage IV, local stage III, completely necrotic Wilms tumours is excellent. Our results suggest that abdominal irradiation in this patient category may not be of added value in first-line treatment, consistent with the current recommendation in the SIOP-RTSG 2016 UMBRELLA protocol.

Project description:BACKGROUND: Current resuscitation guidelines recommend 3:1 Compression:Ventilation (C:V) ratio, however the most effective C:V ratio in newborns remains controversial. We recently demonstrate that if chest compressions (CC) are superimposed by sustained inflations (SI) return of spontaneous circulation (ROSC) and mortality are improved in asphyxiated newborn piglets when compared to standard coordinated 3:1 resuscitation. However, this has not been studied in newborns. OBJECTIVES: To determine if CC superimposed with SI impacts ROSC in infants <33 weeks with bradycardia or asystole compared to coordinated 3:1 C:V resuscitation. DESIGN/METHODS: In a pilot study, infants born <33 weeks gestational age with an heart rate <60/min or asystole were randomized to receive either “3:1 C:V” or “SS+CC”. Infants randomized to 3:1 received coordinated CC and ventilation according to the current resuscitation guidelines. Infants randomized to “SI+CC” received uninterrupted CC at a rate of 90/min superimposed by a SI of 30 sec. The default settings for airway pressures were peak inflation pressure of 24 cm H2O and a positive end expiratory pressure of 6 cm H2O. The primary outcome was duration of CC to achieve ROSC. Deferral of consent was approved by the institutional ethics committee. The trial was registered at ClinicalTrials.gov: NCT02083705. RESULTS: Five infants were randomized to each group; the mean (range) gestational age was 25 (24–27) weeks vs. 26 (23–28) weeks in the in the SI+CC and 3:1 group (P=0.433), respectively. Mean (SD) birth weight in the SI+CC group was 797 (208) g vs. 808 (182) g in the 3:1 group, (P=0.465) respectively. Mean (SD) ROSC was significantly decreased in the SI+CC group with 31 (8) sec vs. 138 (72) sec in the 3:1 group (P=0.011). One infant in each group died and only one infant in the 3:1 group received epinephrine. One infant in the SI+CC group and three in the 3:1 had intracranial hemorrhage grade 3 or greater. Three infants in the SI+CC group had necrotizing enterocolitis vs. one infant in the 3:1 group (P=0.197). CONCLUSION: Preterm infants <33 weeks had significant shorter ROSC when CC were superimposed by SI compared to using 3:1.

Dataset Information

Prognostic significance of age in 5631 patients with Wilms tumour prospectively registered in International Society of Paediatric Oncology (SIOP) 93-01 and 2001.

Publications

Prognostic significance of age in 5631 patients with Wilms tumour prospectively registered in International Society of Paediatric Oncology (SIOP) 93-01 and 2001.

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