Wilms tumor with intravascular extension: Analyzing tumor thrombus characteristics and survival

Abstract

Objective: The aim of the study is to review the oncological and surgical strategies for Wilms tumor (WT) with intravascular thrombus extension at our institution. Patients and Methods: Medical records of patients with WT treated in our institution between June 2012 and June 2024 were retrospectively reviewed. Among these data, patients with intravascular tumor thrombus were identified. Demographics, imaging studies to determine the initial level of thrombus, surgical management and outcomes were analyzed. Results: During this period 51 patients with WT were treated in our institution. Seven patients (6 male, 1 female) with intravascular tumor thrombus extension were identified (13%). The median age was 46 months (22-67 months). Six patients had unilateral WT (UWT) and one patient had bilateral WT (BWT). Tumor thrombus extension during initial evaluation were: level III (5), level IV (1) and level V (1). Thrombus level in all patients with level III regressed to level II, while, no regression was observed in 2 patients with cardiac thrombus. All patients underwent surgery after neoadjuvant chemotherapy. At a median follow-up of 5 years, the 5-year eventfree survival and overall survival rates were 83.3% and 83.3% respectively in patients with intravascular thrombus. In addition, the 5-year event-free survival and overall survival rates were 85% and 94% respectively in patients without intravascular thrombus. Conclusion: Management of WT with intravascular thrombus is a challenging process. Multidisciplinary approach and an experienced surgical team are the main factors that determine survival in these patients.

Keywords

• Wilms tumor
• Intravascular extension
• Children

References

1. Lopyan NM, Ehrlich PF. Surgical management of Wilms tumor (nephroblastoma) and renal cell carcinoma in children and young adults. Surg Oncol Clin N Am 2021;30:305-23. doi:10.1016/j.soc.2020.11.002
2. Aldrink JH, Heaton TE, Dasgupta R, et al. Update on Wilms tumor. J Pediatr Surg 2019;54:390-7.
3. Naik-Mathuria B, Utria AF, Ehrlich PF, et al. Management and outcomes of Wilms tumor with suprarenal intravascular extension: A pediatric surgical oncology research collaborative study. Ann Surg 2024;279:528-35. doi: 10.1097/ SLA.000.000.0000005921
4. Pio L, Abib S, Guerin F, et al. Surgical management of Wilms tumors with intravenous extension: A multicenter analysis of clinical management with technical insights. Ann Surg Oncol 2024;31:4713-23. Available from: https://doi.org/10.1245/ s10434.024.15232-w
5. Szavay P, Luithle T, Semler O, Graf N, Fuchs J. Surgery of cavoatrial tumor thrombus in nephroblastoma: A report of the SIOP/GPOH study. Pediatr Blood Cancer 2004;43:40-5.
6. Khanna G, Rosen N, Anderson JR, et al. Evaluation of diagnostic performance of CT for detection of tumor thrombus in children with Wilms tumor: A report from the Children’s Oncology Group. Pediatr Blood Cancer 2012;58:551-5.
7. Abdullah Y, Karpelowsky J, Davidson A, et al. Management of nine cases of Wilms’ tumour with intracardiac extension: A single centre experience. J Pediatr Surg 2013;48:394-9. doi:10.1016/j.jpedsurg.2012.11.024
8. Cox S, Büyükünal C, Millar AJW. Surgery for the complex Wilms tumour. Pediatr Surg Int 2020;36:113-27. doi: 10.1007/ s00383.019.04596-w

9. Daum R, Roth H. Tumor infiltration of the vena cava in nephroblastoma. Pediatr Surg Int 1992;7:16-20.
10. Bader MI, Abdelaal K, Rogers T, Arul SG. A surgical approach to Wilms’ tumour with retrohepatic vena caval extension. Pediatr Surg Int 2013;29:229-32.
11. Ritchey ML, Kelalis PP, Breslow N, Offord KP, Shochat SJ, D’Angio GJ. Intracaval and atrial involvement with nephroblastoma: Review of National Wilms Tumor Study-3. J Urol 1988;140(5 Pt 2):1113-8. doi: 10.1016/S0022- 5347(17)41975-6
12. Ritchey ML, Pringle KC, Breslow NE, et al. Management and outcome of inoperable Wilms tumor: A report of National Wilms Tumor Study-3. Ann Surg 1994;220:683-90.
13. Shamberger RC, Ritchey ML, Haase GM, et al. Intravascular extension of Wilms tumor. Ann Surg 2001;234:116-21.
14. Elayadi M, Hammad M, Sallam K, et al. Management and outcome of pediatric Wilms tumor with malignant inferior vena cava thrombus: Largest cohort of single-center experience. Int J Clin Oncol 2020;25:1425-31. doi:10.1007/ s10147.020.01667-0
15. Morris L, Squire R, Sznajder B, van Tinteren H, Godzinski J, Powis M. Optimal neoadjuvant chemotherapy duration in Wilms tumour with intravascular thrombus: A literature review and evidence from SIOP WT 2001 trial. Pediatr Blood Cancer 2019;66:e27941.
16. Sutthatarn P, Gomez Quevedo O, Gleason J, Davidoff AM, Murphy AJ. Management of intravascular thrombus in cases of bilateral Wilms tumor or horseshoe kidney. J Pediatr Surg 2022;57:166-73. doi: 10.1016/j.jpedsurg.2021.07.025
17. Qureshi SS, Bhagat M, Smriti V, et al. Intravascular extension of Wilms tumor: Characteristics of tumor thrombus and their impact on outcomes. J Pediatr Urol 2021;17:69.e1-69.e8. doi:10.1016/j.jpurol.2020.10.003
18. Boam TD, Gabriel M, Shukla R, Losty PD. Impact of neoadjuvant chemotherapy on thrombus viability in patients with Wilms tumour and caval extension: Systematic review with meta-analysis. BJS Open 2021;5:zrab036.
19. Dzhuma K, Powis M, Vujanic G, et al. Surgical management, staging, and outcomes of Wilms tumours with intravascular extension: Results of the IMPORT study. J Pediatr Surg 2022;57:572-8. doi: 10.1016/j.jpedsurg.2021.08.023
20. Ehrlich P. Commentary on surgical management, staging, and outcomes of Wilms tumours with intravascular extension: Results of the IMPORT study. J Pediatr Surg 2022;57:579-80. doi:10.1016/j.jpedsurg.2021.09.009
21. Loh A, Bishop M, Krasin M, Davidoff AM, Langham MR. Long-term physiologic and oncologic outcomes of inferior vena cava thrombosis in pediatric malignant abdominal tumors. J Pediatr Surg 2015;50:550-5. doi: 10.1016/j. jpedsurg.2014.11.044