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

Kristina Dzhuma; Mark Powis; Gordan Vujanic; Tom Watson; Oystein Olsen; Susan Shelmerdine; Minou Oostveen; Jesper Brok; Sabine Irtan; Richard Williams; Suzanne Tugnait; Naima Smeulders; Imran Mushtaq; Tanzina Chowdhury; Reem Al-Saadi; Kathy Pritchard-Jones

doi:10.1016/j.jpedsurg.2021.08.023

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

Kristina Dzhuma, Mark Powis, Gordan Vujanic, Tom Watson, Oystein Olsen, Susan Shelmerdine, Minou Oostveen, Jesper Brok, Sabine Irtan, Richard Williams, Suzanne Tugnait, Naima Smeulders, Imran Mushtaq, Tanzina Chowdhury, Reem Al-Saadi, Kathy Pritchard-Jones

Department of Paediatrics and Adolescent Medicine

20 Citations (Scopus)

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.

Original language	English
Journal	Journal of Pediatric Surgery
Volume	57
Issue number	4
Pages (from-to)	572-578
Number of pages	7
ISSN	0022-3468
DOIs	https://doi.org/10.1016/j.jpedsurg.2021.08.023
Publication status	Published - Apr 2022

Keywords

Local stage
Outcomes
Surgery
Thrombus
Wilms tumor

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10.1016/j.jpedsurg.2021.08.023

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Dzhuma, K., Powis, M., Vujanic, G., Watson, T., Olsen, O., Shelmerdine, S., Oostveen, M., Brok, J., Irtan, S., Williams, R., Tugnait, S., Smeulders, N., Mushtaq, I., Chowdhury, T., Al-Saadi, R., & Pritchard-Jones, K. (2022). Surgical management, staging, and outcomes of Wilms tumours with intravascular extension: Results of the IMPORT study. Journal of Pediatric Surgery, 57(4), 572-578. https://doi.org/10.1016/j.jpedsurg.2021.08.023

Dzhuma, K, Powis, M, Vujanic, G, Watson, T, Olsen, O, Shelmerdine, S, Oostveen, M, Brok, J, Irtan, S, Williams, R, Tugnait, S, Smeulders, N, Mushtaq, I, Chowdhury, T, Al-Saadi, R & Pritchard-Jones, K 2022, 'Surgical management, staging, and outcomes of Wilms tumours with intravascular extension: Results of the IMPORT study', Journal of Pediatric Surgery, vol. 57, no. 4, pp. 572-578. https://doi.org/10.1016/j.jpedsurg.2021.08.023

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

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.",

keywords = "Local stage, Outcomes, Surgery, Thrombus, Wilms tumor",

author = "Kristina Dzhuma and Mark Powis and Gordan Vujanic and Tom Watson and Oystein Olsen and Susan Shelmerdine and Minou Oostveen and Jesper Brok and Sabine Irtan and Richard Williams and Suzanne Tugnait and Naima Smeulders and Imran Mushtaq and Tanzina Chowdhury and Reem Al-Saadi and Kathy Pritchard-Jones",

note = "Copyright {\textcopyright} 2021. Published by Elsevier Inc.",

year = "2022",

month = apr,

doi = "10.1016/j.jpedsurg.2021.08.023",

language = "English",

volume = "57",

pages = "572--578",

journal = "Journal of Pediatric Surgery",

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TY - JOUR

T1 - Surgical management, staging, and outcomes of Wilms tumours with intravascular extension

T2 - Results of the IMPORT study

AU - Dzhuma, Kristina

AU - Powis, Mark

AU - Vujanic, Gordan

AU - Watson, Tom

AU - Olsen, Oystein

AU - Shelmerdine, Susan

AU - Oostveen, Minou

AU - Brok, Jesper

AU - Irtan, Sabine

AU - Williams, Richard

AU - Tugnait, Suzanne

AU - Smeulders, Naima

AU - Mushtaq, Imran

AU - Chowdhury, Tanzina

AU - Al-Saadi, Reem

AU - Pritchard-Jones, Kathy

PY - 2022/4

Y1 - 2022/4

N2 - 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.

AB - 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.

KW - Local stage

KW - Outcomes

KW - Surgery

KW - Thrombus

KW - Wilms tumor

UR - https://www.scopus.com/pages/publications/85115748476

U2 - 10.1016/j.jpedsurg.2021.08.023

DO - 10.1016/j.jpedsurg.2021.08.023

M3 - Review

C2 - 34565577

SN - 0022-3468

VL - 57

SP - 572

EP - 578

JO - Journal of Pediatric Surgery

JF - Journal of Pediatric Surgery

IS - 4

ER -

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

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Keywords

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