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Surgical management, staging, and outcomes of Wilms tumours with intravascular extension: Results of the IMPORT study

Dzhuma, K; Powis, M; Vujanic, G; Watson, T; Olsen, O; Shelmerdine, S; Oostveen, M; ... Pritchard-Jones, K; + view all (2022) Surgical management, staging, and outcomes of Wilms tumours with intravascular extension: Results of the IMPORT study. Journal of Pediatric Surgery , 57 (4) pp. 572-578. 10.1016/j.jpedsurg.2021.08.023. Green open access

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Abstract

PURPOSE: To review surgical management, tumour stage and clinical outcomes in children with intravascular extension of Wilms tumour (WT) registered in a national clinical study (2012-19). METHODS: WTs with presence/suspicion of tumour thrombus in the renal vein (RV) or beyond on radiology, surgery or pathology case report forms were identified. Only cases where thrombus was confirmed by surgeon and/or reference pathologist were included. Surgical management, disease stage, overall (OS) and event free survival (EFS) were investigated. RESULTS: 69/583 (11.8%) patients met the inclusion criteria. Forty-six (67%) had abdominal stage III due to thrombus-related reasons: 11 had macroscopically incomplete resection, including 8 cases where cavotomy was not performed; 20 had piecemeal complete resection of thrombus; 15 had microscopically positive resection margins at the RV. 66% of tumour thrombi contained viable tumour. There were eight relapses and five deaths. EFS, but not OS, was significantly associated with completeness of surgical resection (P<0.05). OS and EFS were also significantly associated with histological risk group (P<0.05) but not with viability of tumour thrombus (P=0.19; P=0.59). CONCLUSIONS: WTs with intravascular extension have a high risk of local stage III due to thrombus-related reasons. Controlled complete removal of the thrombus should be the aim of surgery. LEVEL OF EVIDENCE: Level II.

Type: Article
Title: Surgical management, staging, and outcomes of Wilms tumours with intravascular extension: Results of the IMPORT study
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.jpedsurg.2021.08.023
Publisher version: https://doi.org/10.1016/j.jpedsurg.2021.08.023
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Local stage, Outcomes, Surgery, Thrombus, Wilms tumor
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Biology and Cancer Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10135569
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