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How does Nephroblastomatosis impact the prognosis of unilateral Wilms tumor?

  • Urology - Original Paper
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Abstract

Purpose

We aimed to explore whether Nephroblastomatosis (Nbm) has an effect on the prognosis of Wilms tumor (WT), and compare the relapse-free survival (RFS) and overall survival (OS) after surgery of WT patients with or without Nbm.

Methods

We retrospectively analyzed the clinical data of children with WT admitted to our department from April 2010 to September 2021. The enrolled patients were divided into two groups according to whether they were combined with Nbm histologically or not, the Nbm group for patients accompany with Nbm and the non-Nbm group for pure WT. All patients underwent a standardized comprehensive treatment according to National Wilms Tumor Study 5. Clinical variables, pathological results, and the prognosis were collected during follow-up.

Results

A total of 345 patients were finally enrolled in this study, of which 299 patients in the non-Nbm group and 46 patients in the Nbm group. Univariate Cox analysis revealed that Nbm was not the risk factor of either OS or RFS. The difference of postoperative RFS (P = 0.66) and OS (P = 0.68) between two groups was not significant. Subgroup analysis revealed that the RFS and OS between the non-Nbm group and the Nbm group had no statistical difference under the condition of same stage (low grade and high grade), same histology (favorable histology and unfavorable histology), same surgical approach (nephrectomy and nephron-sparing surgery), with or without lymph node metastasis (P > 0.05).

Conclusion

Although Nbm had a strong correlation with the occurrence of WT, a combined Nbm did not increase the risk of metastasis and poor prognosis of WT. After complete surgical resection followed by standardized treatment, the long-term RFS and OS were not different from pure WT.

IEC-C-006-A04-V.06 retrospectively registered.

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Availability of data and materials

The datasets used and/or analyzed during the current study are available from the corresponding authors and first authors on reasonable request.

Abbreviations

Nbm:

Nephroblastomatosis

WT:

Wilms tumor

RFS:

Relapse-free survival

OS:

Overall survival

NRs:

Nephrogenic rests

FH:

Favorable histology

UH:

Unfavorable histology

NSS:

Nephron-sparing surgery

References

  1. Vicens J, Iotti A, Lombardi MG et al (2009) Diffuse hyperplastic perilobar nephroblastomatosis. Pediatr Dev Pathol 12:237–238. https://doi.org/10.2350/07-09-0349.1

    Article  PubMed  Google Scholar 

  2. de Jesus LE, Fulgencio C, Leve TC, Dekermacher S (2022) Nephroblastomatosis and Wilms tumor: dangerous liaisons. Int braz j urol 48:157–164. https://doi.org/10.1590/s1677-5538.ibju.2020.0694

    Article  PubMed  Google Scholar 

  3. Pater L, Melchior P, Rübe C et al (2021) Wilms tumor. Pediatric Blood Cancer. https://doi.org/10.1002/pbc.28257

    Article  PubMed  PubMed Central  Google Scholar 

  4. van den Heuvel-Eibrink MM, Hol JA, Pritchard-Jones K et al (2017) Position paper: rationale for the treatment of Wilms tumour in the UMBRELLA SIOP-RTSG 2016 protocol. Nat Rev Urol 14:743–752. https://doi.org/10.1038/nrurol.2017.163

    Article  PubMed  Google Scholar 

  5. Vanden Berg RNW, Bierman EN, Noord MV et al (2016) Nephron-sparing surgery for Wilms tumor: a systematic review. Urol Oncol 34:24–32. https://doi.org/10.1016/j.urolonc.2015.07.003

    Article  PubMed  Google Scholar 

  6. Green DM (2004) The treatment of stages I-IV favorable histology Wilms’ tumor. J Clin Oncol 22:1366–1372. https://doi.org/10.1200/JCO.2004.08.008

    Article  PubMed  Google Scholar 

  7. Dome JS, Cotton CA, Perlman EJ et al (2006) Treatment of anaplastic histology Wilms’ tumor: results from the fifth National Wilms’ tumor study. J Clin Oncol 24:2352–2358. https://doi.org/10.1200/JCO.2005.04.7852

    Article  PubMed  Google Scholar 

  8. Green DM, Beckwith JB, Breslow NE et al (1994) Treatment of children with stages II to IV anaplastic Wilms’ tumor: a report from the National Wilms’ Tumor study group. J Clin Oncol 12:2126–2131. https://doi.org/10.1200/JCO.1994.12.10.2126

    Article  CAS  PubMed  Google Scholar 

  9. Hou LT, Holman RL (1961) Bilateral nephroblastomatosis in a premature infant. J Pathol Bacteriol 82:249–255. https://doi.org/10.1002/path.1700820202

    Article  CAS  PubMed  Google Scholar 

  10. Beckwith JB, Kiviat NB, Bonadio JF (1990) Nephrogenic rests, nephroblastomatosis, and the pathogenesis of Wilms’ tumor. Pediatr Pathol 10:1–36. https://doi.org/10.3109/15513819009067094

    Article  CAS  PubMed  Google Scholar 

  11. Perlman EJ, Faria P, Soares A et al (2006) Hyperplastic perilobar nephroblastomatosis: long-term survival of 52 patients. Pediatr Blood Cancer 46:203–221. https://doi.org/10.1002/pbc.20386

    Article  PubMed  Google Scholar 

  12. Ehrlich PF, Tornwall B, Chintagumpala MM et al (2022) Kidney preservation and Wilms tumor development in children with diffuse hyperplastic perilobar nephroblastomatosis: a report from the children’s oncology group study AREN0534. Ann Surg Oncol 29:3252–3261. https://doi.org/10.1245/s10434-021-11266-6

    Article  PubMed  Google Scholar 

  13. Bergeron C, Iliescu C, Thiesse P et al (2001) Does nephroblastomatosis influence the natural history and relapse rate in Wilms’ tumour? A single centre experience over 11 years. European J Cancer. https://doi.org/10.1016/S0959-8049(00)00409-3

    Article  Google Scholar 

  14. Sandberg JK, Chi Y-Y, Smith EA et al (2020) Imaging characteristics of nephrogenic rests versus small Wilms tumors: a report from the children’s oncology group study AREN03B2. Am J Roentgenol 214:987–994. https://doi.org/10.2214/AJR.19.22301

    Article  Google Scholar 

  15. Gylys-Morin V, Hoffer FA, Kozakewich H, Shamberger RC (1993) Wilms tumor and nephroblastomatosis: imaging characteristics at gadolinium-enhanced MR imaging. Radiology 188:517–521. https://doi.org/10.1148/radiology.188.2.8392214

    Article  CAS  PubMed  Google Scholar 

  16. Grundy PE, Breslow NE, Li S et al (2005) Loss of heterozygosity for chromosomes 1p and 16q is an adverse prognostic factor in favorable-histology Wilms tumor: a report from the National Wilms tumor study group. J Clin Oncol 23:7312–7321. https://doi.org/10.1200/JCO.2005.01.2799

    Article  CAS  PubMed  Google Scholar 

  17. Graf N, van Tinteren H, Bergeron C et al (2012) Characteristics and outcome of stage II and III non-anaplastic Wilms’ tumour treated according to the SIOP trial and study 93–01. Eur J Cancer 48:3240–3248. https://doi.org/10.1016/j.ejca.2012.06.007

    Article  PubMed  Google Scholar 

  18. Furtwängler R, Schmolze M, Gräber S et al (2014) Pretreatment for bilateral nephroblastomatosis is an independent risk factor for progressive disease in patients with stage V nephroblastoma. Klin Padiatr 226:175–181. https://doi.org/10.1055/s-0034-1371840

    Article  PubMed  Google Scholar 

  19. Williams RD, Chagtai T, Alcaide-German M et al (2015) Multiple mechanisms of MYCN dysregulation in Wilms tumour. Oncotarget 6:7232–7243. https://doi.org/10.18632/oncotarget.3377

    Article  PubMed  PubMed Central  Google Scholar 

  20. Ooms AHAG, Gadd S, Gerhard DS et al (2016) Significance of TP53 mutation in Wilms Tumors with diffuse anaplasia: a report from the children’s oncology group. Clin Cancer Res 22:5582–5591. https://doi.org/10.1158/1078-0432.CCR-16-0985

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Horwitz JR, Ritchey ML, Moksness J et al (1996) Renal salvage procedures in patients with synchronous bilateral Wilms’ tumors: a report from the National Wilms’ tumor study group. J Pediatr Surg 31:1020–1025. https://doi.org/10.1016/s0022-3468(96)90077-9

    Article  CAS  PubMed  Google Scholar 

  22. Cozzi F, Schiavetti A, Cozzi DA et al (2004) Conservative management of hyperplastic and multicentric nephroblastomatosis. J Urol 172:1066–1070. https://doi.org/10.1097/01.ju.0000134883.12993.82

    Article  PubMed  Google Scholar 

  23. Ortiz MV, Fernandez-Ledon S, Ramaswamy K et al (2019) Maintenance chemotherapy to reduce the risk of a metachronous Wilms tumor in children with bilateral nephroblastomatosis. Pediatr Blood Cancer 66:e27500. https://doi.org/10.1002/pbc.27500

    Article  PubMed  Google Scholar 

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Author notes

  1. Xinyu Wang and Jiayi Li have contributed equally and should be considered as the co-first authors.

Authors and Affiliations

  1. Department of Urology, National Center for Children’s Health (NCCH), Beijing Children’s Hospital, Capital Medical University, Beijing, 100045, China

    Xinyu Wang, Jiayi Li, Pei Liu, Jun Tian, Minglei Li, Ning Sun, Weiping Zhang & Hongcheng Song

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Correspondence to Hongcheng Song.

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Wang, X., Li, J., Liu, P. et al. How does Nephroblastomatosis impact the prognosis of unilateral Wilms tumor?. Int Urol Nephrol 55, 803–811 (2023). https://doi.org/10.1007/s11255-023-03493-5

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