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Inferior vena cava involvement in children with Wilms tumor

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Abstract

Objectives

Retrospective review of children with WT thrombus involving the IVC.

Methods

We reviewed the charts of 123 patients with WT diagnosed between January 2006 and December 2015. Patients with caval tumor thrombus were identified, demographic data, radiological images, extent of thrombus, chemo- and radiotherapy, surgical approach, pathology reports and outcomes were analyzed.

Results

IVC involvement was identified in 11 patients (9%). Left-sided tumors were more common (55%). Sensitivity of CT scan in thrombus identification was 64%. Neoadjuvant chemotherapy was the primary mode of treatment with 19% reduction of primary tumor size, thrombus resolution in four and regression in six patients. No thrombus progression was observed. Delayed surgical treatment was either nephro-ureterectomy (nine patients) or partial nephrectomy in patients with bilateral WT (two patients). During surgery, thrombectomy and intimal stripping achieved local control in patients with persistent caval tumor. Cardiopulmonary bypass was not needed in patients with initial atrial involvement (five patients). Despite adherence and invasion of tumor thrombus to IVC wall, only one patient required segmental vein resection. All patients eventually achieved CR, none had local relapse. One patient had lung relapse treated to complete remission. The 3-year EFS is 85.7 ± 13.2% and the 3-year OS is 100%.

Conclusion

IVC tumor thrombus in patients with WT was managed successfully using preoperative chemotherapy followed by surgery with minimal aggressive approach. Luminal thrombus removal and intimal stripping supplanted by local radio boost when indicated seem to be adequate tools for local control with documented good overall outcomes.

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References

  1. Aspiazu D, Fernandez-Pineda I, Cabello R, Ramirez G, Alvarez-Madrid A, De Agustin JC (2012) Surgical management of Wilms tumor with intravascular extension: a single-institution experience. Pediatr Hematol Oncol 29(1):50–54

    Article  CAS  PubMed  Google Scholar 

  2. Hadley GP, Sheik-Gafoor MH, Buckels NJ (2010) The management of nephroblastoma with cavo-atrial disease at presentation: experience from a developing country. Pediatr Surg Int 26:1169–1172

    Article  CAS  PubMed  Google Scholar 

  3. Ritchey ML, Othersen HB Jr, de Lorimier AA, Kramer SA, Benson C, Kelalis PP (1989) Renal vein involvement with nephroblastoma: a report of the National Wilms’ Tumor Study-3. Eur Urol 17(2):139–144

    Google Scholar 

  4. Khanna G, Rosen N, Anderson JR, Ehrlich PF, Dome JS, Gow KW, Grundy P (2012) 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 58(4):551–555

    Article  PubMed  Google Scholar 

  5. MedCalc Statistical Software version 15.8 (MedCalc Software bvba) (2015) Ostend, Belgium. https://www.medcalc.org

  6. Hinman F (1998) Atlas of urologic surgery. W.B. Saunders Co, Philadelphia

    Google Scholar 

  7. Shamberger RC, Ritchey ML, Haase GM, Bergemann TL, Loechelt-Yoshioka T, Breslow NE, Green DM (2001) Intravascular extension of Wilms tumor. Ann Surg 234(1):116

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Ritchey ML, Kelalis PP, Breslow N, Offord KP, Shochat SJ, D’Angio GJ (1988) Intracaval and atrial involvement with nephroblastoma: review of National Wilms Tumor Study-3. J Urol 140(5 Pt 2):1113–1118

    CAS  PubMed  Google Scholar 

  9. Akyüz C, Emir S, Büyükpamukçu N et al (2005) Cavoatrial tumor extension in children with Wilms tumor: a retrospective review of 17 children in a single center. J Pediatr Hematol Oncol 27:267–269

    Article  PubMed  Google Scholar 

  10. Szavay P, Luithle T, Semler O et al (2004) Surgery of cavoatrial tumor thrombus in nephroblastoma: a report of the SIOP/GPOH. Pediatr Blood Cancer 43:40–45

    Article  PubMed  Google Scholar 

  11. Federici S, Galli G, Ceccarelli PL et al (1994) Wilms’ tumor involving the inferior vena cava: preoperative evaluation and management. Med Pediatr Oncol 22:39–44

    Article  CAS  PubMed  Google Scholar 

  12. Nakayama D, Delorimier A, O’Neill J, Norkool P, D’Angio G (1986) Intracardiac extension of Wilms’ tumor. A report of the National Wilms’ Tumor Study. Ann Surg 204:693

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Martinez-Ibanez V, de Toledo JS, De Diego M et al (1996) Wilms’ tumours with intracaval involvement. Med Pediatr Oncol 26:268–271

    Article  CAS  PubMed  Google Scholar 

  14. Brisse HJ, Smets AM, Kaste SC et al (2008) Imaging in unilateral Wilms tumour. Pediatr Radiol 38(1):18–29

    Article  PubMed  Google Scholar 

  15. Nakamura L, Ritchey M (2010) Current management of wilms’ tumor. Curr Urol Rep 11(1):58–65

    Article  PubMed  Google Scholar 

  16. Davidoff AM (2009) Wilms’ tumor. Curr Opin Pediatr 21(3):357–364

    Article  PubMed  PubMed Central  Google Scholar 

  17. Ritchey ML, Kelalis PP, Haase GM, Shochat SJ, Green DM, D’Angio G (1993) Preoperative therapy for intracaval and atrial extension of Wilms tumor. Cancer 71(12):4104–4110

    Article  CAS  PubMed  Google Scholar 

  18. Cristofani LM, Duarte RJ, Almeida MT et al (2007) Intracaval and intracardiac extension of Wilms tumor. The influence of preoperative chemotherapy on surgical morbidity. Int Braz J Urol 33:683–689

    Article  PubMed  Google Scholar 

  19. Mushtaq I, Carachi R, Roy G et al (1996) Childhood renal tumours with intravascular extension. Br J Urol 78:772–776

    Article  CAS  PubMed  Google Scholar 

  20. Giannoulia-Karadana A, Moschovi M, Koutsovitis P, Tolis G, Tzortzatou-Stathopoulou F (2000) Inferior vena cava and right atrial thrombosis in children with nephroblastoma: diagnostic and therapeutic problems. J Pediatr Surg 35:1459–1461

    Article  CAS  PubMed  Google Scholar 

  21. Loh A, Bishop M, Krasin M, Davidoff AM, Langham MR (2015) Long-term physiologic and oncologic outcomes of inferior vena cava thrombosis in pediatric malignant abdominal tumors. J Pediatr Surg 50(4):550–555

    Article  PubMed  Google Scholar 

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Correspondence to Abeer Al Diab.

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Al Diab, A., Hirmas, N., Almousa, A. et al. Inferior vena cava involvement in children with Wilms tumor. Pediatr Surg Int 33, 569–573 (2017). https://doi.org/10.1007/s00383-016-4034-7

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