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Surgical Management of Wilms Tumors with Intravenous Extension: A Multicenter Analysis of Clinical Management with Technical Insights

  • Pediatric Oncology
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

About 5% of Wilms tumors present with vascular extension, which sometimes extends to the right atrium. Vascular extension does not affect the prognosis, but impacts the surgical strategy, which is complex and not fully standardized. Our goal is to identify elements of successful surgical management of Wilms tumors with vascular extensions.

Patients and Methods

A retrospective study of pediatric Wilms tumors treated at three sites (January 1999–June 2019) was conducted. The inclusion criterion was the presence of a renal vein and vena cava thrombus at diagnosis. Tumor stage, pre and postoperative treatment, preoperative imaging, operative report, pathology, operative complications, and follow-up data were reviewed.

Results

Of the 696 pediatric patients with Wilms tumors, 69 (9.9%) met the inclusion criterion. In total, 24 patients (37.5%) had a right atrial extension and two presented with Budd–Chiari syndrome at diagnosis. Two died at diagnosis owing to pulmonary embolism. All patients received neoadjuvant chemotherapy and thrombus regressed in 35.6% of cases. Overall, 14 patients had persistent intra-atrial thrombus extension (58%) and underwent cardiopulmonary bypass. Most thrombi (72%) were removed intact with nephrectomy. Massive intraoperative bleeding occurred during three procedures. Postoperative renal insufficiency was identified as a risk factor for patient survival (p = 0.01). With a median follow-up of 9 years (range: 0.5–20 years), overall survival was 89% and event-free survival was 78%.

Conclusions

Neoadjuvant chemotherapy with proper surgical strategy resulted in a survival rate comparable to that of children with Wilms tumors without intravascular extension. Clinicians should be aware that postoperative renal insufficiency is associated with worse survival outcomes.

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References

  1. Lall A, Pritchard-Jones K, Walker J, et al. Wilms’ tumor with intracaval thrombus in the UK Children’s Cancer Study Group UKW3 trial. J Pediatr Surg. 2006;41:382–7.

    Article  PubMed  Google Scholar 

  2. Ritchey ML, Pringle KC, Breslow NE, et al. Management and outcome of inoperable wilms’ tumour. A report of National Wilms’ Tumour Study-3. Ann Surg. 1994;220:683–90.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Abdullah Y, Karpelowsky J, Davidson A, et al. Management of nine cases of Wilms’ tumor with intra cardiac extension — a single centre experience. J Pediatr Surg. 2003;48:394–9.

    Article  Google Scholar 

  4. Nakayama DK, Norkool P, deLorimier AA, et al. Intracardiac extension of Wilms’ tumour. A report of the National Wilms’ Tumour Study. Ann Surg. 1986;204:693–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Zavay P, Luithie 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.

    Article  Google Scholar 

  6. Mushtaq I, Carachi R, Roy G, Azmy A. Childhood renal tumours with intravascular extension. Br J Urol. 1996;78:772–6.

    Article  CAS  PubMed  Google Scholar 

  7. Agoustides JG, Weiss SJ, Ochroch AE, et al. Analysis of the interatrial septum by transesophageal echocardiography in adult cardiac surgical patients: Anatomic variants and correlation with patent foramen ovale. J Cardiothorac Vasc Anesth. 2005;19:146–9.

    Article  Google Scholar 

  8. Agoustides JG, Weiss SJ, Weiner J, et al. Diagnosis of patent foramen ovale with multiplane transesophageal echocardiography in adult cardiac surgical patients. J Cardiothorac Vasc Anesth. 2004;18:725–30.

    Article  Google Scholar 

  9. Morris L, Squire R, Sznajder B, et al. Optimal neoadjuvant chemotherapy duration in Wilms tumour with intravascular thrombus: a literature review and evidence from SIOP WT 2001 trial. Pediatr Blood Cancer. 2019;24:e27930.

    Article  Google Scholar 

  10. 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(9):166–73.

    Article  PubMed  Google Scholar 

  11. Fanelli MCA, Guilhen JCS, Duarte AAB, de Souza FKM, Cypriano MDS, Caran EMM, Lederman HM, de Seixas Alves MT, Abib SCV. management of pediatric tumors with vascular extension. Front Pediatr. 2022;4(9):753232.

    Article  Google Scholar 

  12. Meier CM, Furtwängler R, von Schweinitz D, Stein R, Welter N, Wagenpfeil S, Kager L, Schenk JP, Vokuhl C, Melchior P, Fuchs J, Graf N. Vena cava thrombus in patients with Wilms tumor. Cancers (Basel). 2022;14(16):3924.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Shamberger RC, Ritchey ML, Haase GM, et al. Intravascular extension of Wilms tumor. Ann Surg. 2001;234:116–21.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. 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. Br J Surg Open. 2021;5(3):zrab020.

    Google Scholar 

  15. Daum R, Roth H, Zachariou Z. Tumour infiltration of the vena cava in nephroblastoma. Eur J Pediatr Surg. 1994;4:16–20.

    Article  CAS  PubMed  Google Scholar 

  16. 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. Surgical management, staging, and outcomes of Wilms tumours with intravascular extension: results of the IMPORT study. J Pediatr Surg. 2022;57(4):572–8.

    Article  PubMed  Google Scholar 

  17. 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. 2023. https://doi.org/10.1097/SLA.0000000000005921.

    Article  PubMed  Google Scholar 

  18. McMahon S, Carachi R. Wilms’ tumor with intravascular extension: a review article. J Indian Assoc Pediatr Surg. 2014;19(4):195–200.

    Article  PubMed  PubMed Central  Google Scholar 

  19. Fukuda A, Isota T, Sakamoto N, et al. Lessons from a patient with cardiac arrest due to massive pulmonary embolism as the initial presentation of Wilms tumor: a case report and literature review. BMC Pediatr. 2019;19(1):39.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Van den Heuvel-Eibrink MM, Lankhorst B, et al. Sudden death due to pulmonary embolism as presenting symptom of renal tumors. Pediatr Blood Cancer. 2008;50(5):1062–4.

    Article  PubMed  Google Scholar 

  21. Wypij D, Newburger JW, Rappaport LA, et al. The effect of duration of deep hypothermic circulatory arrest in infant heart surgery on late neurodevelopment: the Boston Circulatory Arrest Trial. J Thorac Cardiovasc Surg. 2003;126:1397e403.

    Article  Google Scholar 

  22. Lee AY. Anticoagulation in the treatment of established venous thromboembolism in patients with cancer. J Clin Oncol. 2009;27(29):4895–901.

    Article  CAS  PubMed  Google Scholar 

  23. Key NS, Khorana AA, Kuderer NM, et al. Venous thromboembolism prophylaxis and treatment in patients with cancer: ASCO guideline update. J Clin Oncol. 2023;41(16):3063–71.

    Article  CAS  PubMed  Google Scholar 

  24. Khozeimeh N, Sinha P, Dome JS, et al. Strategy for management of retroperitoneal tumors with caval tumor thrombus. J Pediatr Surg. 2011;46(11):2065–70.

    Article  PubMed  Google Scholar 

  25. Romero W, Loockhart A, Moreno D, Pedraza M, Aparicio S, Neira MJ, Cabrera F, Rodriguez M. Suprahepatic cava thrombus due to Wilms tumor: technique without cardiopulmonary bypass. Urology. 2022;165:e25–8.

    Article  PubMed  Google Scholar 

  26. Svensson LG, Crawford ES, Hess KR, et al. Deep hypothermia with circulatory arrest. Determinants of stroke and early mortality in 656 patients. J Thorac Cardiovasc Surg. 1993;106:19e31.

    Article  Google Scholar 

  27. Bader MI, Abdelaal K, Rogers T, Arul SG. A surgical approach to Wilms’ tumour with retrohepatic vena caval extension. Pediatr Surg Int. 2013;29(3):229–32.

    Article  PubMed  Google Scholar 

  28. Ribeiro RC, Schettini ST, Abib Sde C, et al. Cavectomy for the treatment of Wilms tumor with vascular extension. J Urol. 2006;176(1):279–83.

    Article  PubMed  Google Scholar 

  29. Schettini ST, da Fonseca JH, Abib SC, et al. Management of Wilms’ tumor with intracardiac extension. Pediatr Surg Int. 2000;16(7):529–32.

    Article  CAS  PubMed  Google Scholar 

  30. Murthi GV, Kocyildirim E, Sellathury S, et al. Wilms’ tumour with persistent intravascular extension: a review of the surgical aspects of management. J Pediatric Urol. 2006;2:439e445.

    Article  Google Scholar 

  31. Ruiz CS, Kalbaugh CA, Browder SE, et al. Operative strategies for inferior vena cava repair in oncologic surgery. J Vasc Surg Venous Lymphat Disord. 2020;8(3):396–404.

    Article  PubMed  Google Scholar 

  32. Loh A, Bishop M, Krasin M, et al. Long-term physiologic and oncologic outcomes of inferior vena cava thrombosis in pediatric malignant abdominal tumors. J Pediatr Surg. 2015;50(4):550–5.

    Article  PubMed  Google Scholar 

  33. Knijnenburg SL, Mulder RL, Schouten-Van Meeteren AY, et al. Early and late renal adverse effects after potentially nephrotoxic treatment for childhood cancer. Cochrane Database Syst Rev. 2013. https://doi.org/10.1002/14651858.CD008944.pub2.

    Article  PubMed  Google Scholar 

  34. Beck J, Loron G, Masson C, et al. Monitoring cerebral and renal oxygenation status during neonatal digestive surgeries using near infrared spectroscopy. Front Pediatr. 201 Significant neonatal intraoperative cerebral and renal oxygen desaturation identified with near-infrared spectroscopy.

  35. Miyake Y, Seo S, Kataoka K, et al. Significant neonatal intraoperative cerebral and renal oxygen desaturation identified with near-infrared spectroscopy. Pediatr Surg Int. 2022;38(5):737–42.

    Article  PubMed  Google Scholar 

  36. van Peer SE, Hol JA, van der Steeg AFW, Grotel MV, et al. Bilateral renal tumors in children: the first 5 years’ experience of national centralization in the Netherlands and a narrative review of the literature. J Clin Med. 2021;10(23):5558.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Roy P, van Peer SE, de Witte MM, et al. Characteristics and outcome of children with renal tumors in the Netherlands: the first five-year’s experience of national centralization. PLoS ONE. 2022;17(1):e0261729.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Bicêtre Hospital, Paris-Saclay University, GHU Paris Saclay Assistance Publique Hôpitaux de Paris (AP-HP), Le Kremlin Bicêtre, France

    Luca Pio MD, Florent Guerin MD, PhD, Nadia Sarrai MD, Helene Martelli MD, PhD, Frederic Gauthier MD, PhD & Sophie Branchereau MD, PhD

  2. Department of Surgery, St. Jude Children’s Research Hospital, Memphis, TN, USA

    Luca Pio MD

  3. Department of Surgery, Federal University of São Paulo (UNIFESP), São Paulo, Brazil

    Simone Abib MD, PhD, Fernanda K. M. De Souza MD & Mayara C. A. Fanelli MD

  4. Pediatric Oncology Institute—GRAACC—Federal University of São Paulo (UNIFESP), São Paulo, Brazil

    Simone Abib MD, PhD, Fernanda K. M. De Souza MD & Mayara C. A. Fanelli MD

  5. Department of Pediatric Surgery, Hôpital Necker Enfants Malades-Université de Paris Cité, Paris, France

    Christophe Chardot MD, PhD, Thomas Blanc MD, PhD, Julien Rod MD, PhD, Sabine Irtan MD, PhD & Sabine Sarnacki MD, PhD

  6. Department of Cardiovascular Surgery, Hôpital Universitaire Necker Enfants Malades-Université de Paris Cité, Paris, France

    Daniel Tamisier MD

  7. Department of Cardiovascular Surgery, Federal University of São Paulo (UNIFESP), São Paulo, Brazil

    José Cícero S. Guilhen MD

  8. Department of Pediatric Cardiology and Congenital Heart Disease, Hôpital Marie Lannelongue-Groupe Hospitalier Paris-Saclay, Université Paris-Saclay, Le Plessis-Robinson, France

    Emmanuel Le Bret MD, Emré Belli MD & Elie Fadel MD

  9. Pediatric Oncology, Pediatric Oncology Institute—GRAACC—Federal University of São Paulo (UNIFESP), São Paulo, Brazil

    Monica D. S. Cypriano MD

  10. Pediatric Oncology Unit, Institut Gustave Roussy, Paris, France

    Véronique Minard MD, PhD & Claudia Pasqualini MD, PhD

  11. Pediatric Oncology Unit, Institut Curie, Paris, France

    Gudrun Schleiermacher MD, PhD & Lauriane Lemelle MD

  12. Epidemiology, and Biostatistics Unit, Instituto Giannina Gaslini, Genoa, Italy

    Angela Pistorio MD

  13. Paris Kids Cancer, Paris, France

    Luca Pio MD, Florent Guerin MD, PhD, Christophe Chardot MD, PhD, Thomas Blanc MD, PhD, Véronique Minard MD, PhD, Claudia Pasqualini MD, PhD, Gudrun Schleiermacher MD, PhD, Lauriane Lemelle MD, Sabine Irtan MD, PhD, Sophie Branchereau MD, PhD & Sabine Sarnacki MD, PhD

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  1. Luca Pio MD
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  15. Monica D. S. Cypriano MD
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Correspondence to Luca Pio MD.

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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). https://doi.org/10.1245/s10434-024-15232-w

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