Skip to main content
Top

Open Access 07-02-2025 | Kidney Cancer | Review

Paediatric Renal Tumors: A State-of-the-Art Review

Authors: Cecilia Salzillo, Gerardo Cazzato, Gabriella Serio, Andrea Marzullo

Published in: Current Oncology Reports

Login to get access

Abstract

Purpose of Review

Pediatric renal tumors comprise a wide range of conditions, both malignant and benign, that affect children and require a multidisciplinary approach for optimal diagnosis and treatment. This review offers an in-depth analysis of the epidemiology, diagnosis, treatment options, outcomes, and survival of major pediatric renal tumors.

Recent Findings

Wilms tumor, or nephroblastoma, is the most common form of renal tumor in children, characterized by growth from primitive renal cells. Standard treatment involves a combination of surgery, chemotherapy and, in some cases, radiation therapy, with the aim of removing the tumor, preventing recurrence and maximizing the chances of long-term recovery. Less common pediatric renal tumors, such as renal clear cell sarcoma, renal cell carcinoma, mesoblastic nephroma, and malignant rhabdoid tumor, require similarly careful and individualized management. Therapeutic strategies, which depend on the characteristics of the tumor, the stage of the disease and the individual response to therapy, may include surgery, chemotherapy, radiotherapy and, in some cases, molecular targeted therapies, immunotherapies and genetic and epigenetic therapies.

Summary

The management of pediatric kidney tumors requires the involvement of a multidisciplinary team of specialists to ensure accurate evaluation, optimal treatments and long-term follow-up. The aim is to maximize the prospects for recovery and improve the quality of life of patients and their families. Advances in innovative, personalized therapies represent an important opportunity to further improve clinical outcomes in these patients.
Literature
1.
go back to reference Libes J, Hol J, Neto JCA, Vallance KL, Tinteren HV, Benedetti DJ, Villar GLR, Duncan C, Ehrlich PF. Pediatric renal tumor epidemiology: global perspectives, progress, and challenges. Pediatric blood & cancer. 2023, 70 Suppl 2, e30343. Libes J, Hol J, Neto JCA, Vallance KL, Tinteren HV, Benedetti DJ, Villar GLR, Duncan C, Ehrlich PF. Pediatric renal tumor epidemiology: global perspectives, progress, and challenges. Pediatric blood & cancer. 2023, 70 Suppl 2, e30343.
2.
go back to reference Nakata K, Colombet M, Stiller CA, Pritchard-Jones K, Steliarova-Foucher E, Contributors I. Incidence of childhood renal tumours: an international population-based study. Int J Cancer. 2020;147(12):3313–27.PubMedPubMedCentralCrossRef Nakata K, Colombet M, Stiller CA, Pritchard-Jones K, Steliarova-Foucher E, Contributors I. Incidence of childhood renal tumours: an international population-based study. Int J Cancer. 2020;147(12):3313–27.PubMedPubMedCentralCrossRef
3.
go back to reference Qureshi SS, Bhagat M, Verma K, et al. Incidence, treatment, and outcomes of primary and recurrent non-wilms renal tumors in children: report of 109 patients treated at a single institution. J Pediatr Urol. 2020;16(4):e4751–9.CrossRef Qureshi SS, Bhagat M, Verma K, et al. Incidence, treatment, and outcomes of primary and recurrent non-wilms renal tumors in children: report of 109 patients treated at a single institution. J Pediatr Urol. 2020;16(4):e4751–9.CrossRef
4.
go back to reference Jain J, Sutton KS, Hong AL. Progress Update in Pediatric Renal tumors. Curr Oncol Rep. 2021;23(3):33.PubMedCrossRef Jain J, Sutton KS, Hong AL. Progress Update in Pediatric Renal tumors. Curr Oncol Rep. 2021;23(3):33.PubMedCrossRef
5.
go back to reference Vujanić GM, Gessler M, Ooms AHAG, Collini P, Coulomb-l’Hermine A, D’Hooghe E, de Krijger RR, Perotti D, Pritchard-Jones K, Vokuhl C, van den Heuvel-Eibrink MM, Graf N, Study Group (SIOP–RTSG). The UMBRELLA SIOP-RTSG 2016 Wilms tumour pathology and molecular biology protocol. Nat Rev Urol. 2018;15(11):693–701. International Society of Paediatric Oncology–Renal Tumour.PubMedPubMedCentralCrossRef Vujanić GM, Gessler M, Ooms AHAG, Collini P, Coulomb-l’Hermine A, D’Hooghe E, de Krijger RR, Perotti D, Pritchard-Jones K, Vokuhl C, van den Heuvel-Eibrink MM, Graf N, Study Group (SIOP–RTSG). The UMBRELLA SIOP-RTSG 2016 Wilms tumour pathology and molecular biology protocol. Nat Rev Urol. 2018;15(11):693–701. International Society of Paediatric Oncology–Renal Tumour.PubMedPubMedCentralCrossRef
6.
go back to reference Ward E, DeSantis C, Robbins A, Kohler B, Jemal A. Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin. 2014;64(2):83–103.PubMedCrossRef Ward E, DeSantis C, Robbins A, Kohler B, Jemal A. Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin. 2014;64(2):83–103.PubMedCrossRef
7.
go back to reference Schulpen M, Roy P, Wijnen MHWA, Tytgat GAM, van den Heuvel-Eibrink MM, van Tinteren H, Karim-Kos HE. Incidence and survival of paediatric renal tumours in the Netherlands between 1990 and 2014. Eur J cancer (Oxford England: 1990). 2022;175:282–90.CrossRef Schulpen M, Roy P, Wijnen MHWA, Tytgat GAM, van den Heuvel-Eibrink MM, van Tinteren H, Karim-Kos HE. Incidence and survival of paediatric renal tumours in the Netherlands between 1990 and 2014. Eur J cancer (Oxford England: 1990). 2022;175:282–90.CrossRef
8.
go back to reference Leslie SW, Sajjad H, Murphy PB. Wilms Tumor. In StatPearls. StatPearls Publishing. 2023. Leslie SW, Sajjad H, Murphy PB. Wilms Tumor. In StatPearls. StatPearls Publishing. 2023.
9.
go back to reference Behjati S, Gilbertson RJ, Pfister SM. Maturation block in childhood cancer. Cancer Discov. 2021;11:542–4.PubMedCrossRef Behjati S, Gilbertson RJ, Pfister SM. Maturation block in childhood cancer. Cancer Discov. 2021;11:542–4.PubMedCrossRef
10.
go back to reference Spreafico F, Fernandez CV, Brok J, Nakata K, Vujanic G, Geller JI, Gessler M, Maschietto M, Behjati S, Polanco A, Paintsil V, Luna-Fineman S, Pritchard-Jones K. Wilms tumour. Nat Reviews Disease Primers. 2021;7(1):75.PubMedCrossRef Spreafico F, Fernandez CV, Brok J, Nakata K, Vujanic G, Geller JI, Gessler M, Maschietto M, Behjati S, Polanco A, Paintsil V, Luna-Fineman S, Pritchard-Jones K. Wilms tumour. Nat Reviews Disease Primers. 2021;7(1):75.PubMedCrossRef
13.
go back to reference Kitagawa K, Gonoi R, Tatsumi M, Kadowaki M, Katayama T, Hashii Y, Fujisawa M, Shirakawa T. Preclinical Development of a WT1 oral Cancer vaccine using a bacterial Vector to treat castration-resistant prostate Cancer. Mol Cancer Ther. 2019;18(5):980–90.PubMedCrossRef Kitagawa K, Gonoi R, Tatsumi M, Kadowaki M, Katayama T, Hashii Y, Fujisawa M, Shirakawa T. Preclinical Development of a WT1 oral Cancer vaccine using a bacterial Vector to treat castration-resistant prostate Cancer. Mol Cancer Ther. 2019;18(5):980–90.PubMedCrossRef
14.
go back to reference Gadd S, Huff V, Walz AL, Ooms AHAG, Armstrong AE, Gerhard DS, Smith MA, Auvil JMG, Meerzaman D, Chen QR, Hsu CH, Yan C, Nguyen C, Hu Y, Hermida LC, Davidsen T, Gesuwan P, Ma Y, Zong Z, Mungall AJ, Perlman EJ. A children’s Oncology Group and TARGET initiative exploring the genetic landscape of Wilms tumor. Nat Genet. 2017;49(10):1487–94.PubMedPubMedCentralCrossRef Gadd S, Huff V, Walz AL, Ooms AHAG, Armstrong AE, Gerhard DS, Smith MA, Auvil JMG, Meerzaman D, Chen QR, Hsu CH, Yan C, Nguyen C, Hu Y, Hermida LC, Davidsen T, Gesuwan P, Ma Y, Zong Z, Mungall AJ, Perlman EJ. A children’s Oncology Group and TARGET initiative exploring the genetic landscape of Wilms tumor. Nat Genet. 2017;49(10):1487–94.PubMedPubMedCentralCrossRef
15.
go back to reference Nakata K, Colombet M, Stiller CA, Pritchard-Jones K, Steliarova-Foucher E. Incidence of childhood renal tumours: an international population-based study. Int J Cancer. 2020;147:3313–27.PubMedPubMedCentralCrossRef Nakata K, Colombet M, Stiller CA, Pritchard-Jones K, Steliarova-Foucher E. Incidence of childhood renal tumours: an international population-based study. Int J Cancer. 2020;147:3313–27.PubMedPubMedCentralCrossRef
17.
go back to reference Parkin DM, et al. Stage at diagnosis and survival by stage for the leading childhood cancers in three populations of sub-saharan Africa. Int J Cancer. 2021;148:2685–91.PubMedCrossRef Parkin DM, et al. Stage at diagnosis and survival by stage for the leading childhood cancers in three populations of sub-saharan Africa. Int J Cancer. 2021;148:2685–91.PubMedCrossRef
18.
go back to reference Saltzman AF, Cost NG, Romao RL. P. Wilms Tumor. Urologic Clin North Am. 2023;50(3):455–64.CrossRef Saltzman AF, Cost NG, Romao RL. P. Wilms Tumor. Urologic Clin North Am. 2023;50(3):455–64.CrossRef
19.
go back to reference Popov SD, Sebire NJ, Vujanic GM. Wilms’ Tumour– Histology and Differential diagnosis. In: van den Heuvel-Eibrink MM, editor. Wilms Tumor. Codon; 2016. Popov SD, Sebire NJ, Vujanic GM. Wilms’ Tumour– Histology and Differential diagnosis. In: van den Heuvel-Eibrink MM, editor. Wilms Tumor. Codon; 2016.
20.
go back to reference Breslow NE, Beckwith JB, Perlman EJ, Reeve AE. Age distributions, birth weights, nephrogenic rests, and heterogeneity in the pathogenesis of Wilms tumor. Pediatr Blood Cancer. 2006;47(3):260–7.PubMedPubMedCentralCrossRef Breslow NE, Beckwith JB, Perlman EJ, Reeve AE. Age distributions, birth weights, nephrogenic rests, and heterogeneity in the pathogenesis of Wilms tumor. Pediatr Blood Cancer. 2006;47(3):260–7.PubMedPubMedCentralCrossRef
21.
go back to reference Vujanić GM, Sandstedt B. The pathology of Wilms’ tumour (nephroblastoma): the International Society of Paediatric Oncology approach. J Clin Pathol. 2010;63(2):102–9.PubMedCrossRef Vujanić GM, Sandstedt B. The pathology of Wilms’ tumour (nephroblastoma): the International Society of Paediatric Oncology approach. J Clin Pathol. 2010;63(2):102–9.PubMedCrossRef
22.
go back to reference Vujanić GM, Mifsud W. Anaplasia in Wilms tumor: a critical review. Pediatr Blood Cancer. 2024;71(7):e31000.PubMedCrossRef Vujanić GM, Mifsud W. Anaplasia in Wilms tumor: a critical review. Pediatr Blood Cancer. 2024;71(7):e31000.PubMedCrossRef
23.
go back to reference Vujanić GM, Mifsud W, Chowdhury T, Al-Saadi R, Pritchard-Jones K. Renal Tumour Special Interest Group of the children’s Cancer and Leukaemia Group. Characteristics and outcomes of preoperatively treated patients with anaplastic Wilms tumors registered in the UK SIOP-WT-2001 and IMPORT study cohorts (2002–2020). Cancer. 2022;128(8):1666–75.PubMedCrossRef Vujanić GM, Mifsud W, Chowdhury T, Al-Saadi R, Pritchard-Jones K. Renal Tumour Special Interest Group of the children’s Cancer and Leukaemia Group. Characteristics and outcomes of preoperatively treated patients with anaplastic Wilms tumors registered in the UK SIOP-WT-2001 and IMPORT study cohorts (2002–2020). Cancer. 2022;128(8):1666–75.PubMedCrossRef
24.
go back to reference PDQ Pediatric Treatment Editorial Board. Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Health Professional Version. PDQ Cancer Information Summaries. National Cancer Institute (US); 2024. PDQ Pediatric Treatment Editorial Board. Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Health Professional Version. PDQ Cancer Information Summaries. National Cancer Institute (US); 2024.
25.
go back to reference Nelson MV, van den Heuvel-Eibrink MM, Graf N, Dome JS. New approaches to risk stratification for Wilms tumor. Curr Opin Pediatr. 2021;33(1):40–8.PubMedCrossRef Nelson MV, van den Heuvel-Eibrink MM, Graf N, Dome JS. New approaches to risk stratification for Wilms tumor. Curr Opin Pediatr. 2021;33(1):40–8.PubMedCrossRef
26.
go back to reference Pasam MK, Rao BV, Chaganty SK, Sharma RM, Patil V, Kodandapani S, Challa S, Thammineedi SR. Grossing to reporting of Wilms tumor with emphasis on proper sampling in treatment-naive and postchemotherapy specimens and their clinicopathological correlation with outcome. Urol Annals. 2024;16(1):87–93.CrossRef Pasam MK, Rao BV, Chaganty SK, Sharma RM, Patil V, Kodandapani S, Challa S, Thammineedi SR. Grossing to reporting of Wilms tumor with emphasis on proper sampling in treatment-naive and postchemotherapy specimens and their clinicopathological correlation with outcome. Urol Annals. 2024;16(1):87–93.CrossRef
27.
go back to reference Mergen M, Welter N, Furtwängler R, Melchior P, Vokuhl C, Gessler M, Meier CM, Kager L, Schenk JP, Graf N. The impact of the route to diagnosis in nephroblastoma. Cancer medicine. 2024; 13(10), e7226. Mergen M, Welter N, Furtwängler R, Melchior P, Vokuhl C, Gessler M, Meier CM, Kager L, Schenk JP, Graf N. The impact of the route to diagnosis in nephroblastoma. Cancer medicine. 2024; 13(10), e7226.
28.
go back to reference Grundy PE, Breslow NE, Li S, Perlman E, Beckwith JB, Ritchey ML, Shamberger RC, Haase GM, D’Angio GJ, Donaldson M, Coppes MJ, Malogolowkin M, Shearer P, Thomas PR, Macklis R, Tomlinson G, Huff V, Green DM. & National Wilms Tumor Study Group. 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 Oncology: Official J Am Soc Clin Oncol. 2005;23(29):7312–21.CrossRef Grundy PE, Breslow NE, Li S, Perlman E, Beckwith JB, Ritchey ML, Shamberger RC, Haase GM, D’Angio GJ, Donaldson M, Coppes MJ, Malogolowkin M, Shearer P, Thomas PR, Macklis R, Tomlinson G, Huff V, Green DM. & National Wilms Tumor Study Group. 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 Oncology: Official J Am Soc Clin Oncol. 2005;23(29):7312–21.CrossRef
29.
go back to reference Daw NC, Chi YY, Kalapurakal JA, et al. Activity of vincristine and irinotecan in diffuse Anaplastic Wilms Tumor and Therapy outcomes of Stage II to IV Disease: results of the children’s Oncology Group AREN0321 Study. J Clin Oncol. 2020;38(14):1558–68.PubMedPubMedCentralCrossRef Daw NC, Chi YY, Kalapurakal JA, et al. Activity of vincristine and irinotecan in diffuse Anaplastic Wilms Tumor and Therapy outcomes of Stage II to IV Disease: results of the children’s Oncology Group AREN0321 Study. J Clin Oncol. 2020;38(14):1558–68.PubMedPubMedCentralCrossRef
30.
go back to reference Dix DB, Seibel NL, Chi YY, et al. Treatment of Stage IV Favorable Histology Wilms Tumor with Lung metastases: a Report from the children’s Oncology Group AREN0533 Study. J Clin Oncol. 2018;36(16):1564–70.PubMedPubMedCentralCrossRef Dix DB, Seibel NL, Chi YY, et al. Treatment of Stage IV Favorable Histology Wilms Tumor with Lung metastases: a Report from the children’s Oncology Group AREN0533 Study. J Clin Oncol. 2018;36(16):1564–70.PubMedPubMedCentralCrossRef
31.
go back to reference Dome JS, Cotton CA, Perlman EJ, et al. Treatment of anaplastic histology Wilms’ tumor: results from the fifth National Wilms’ Tumor Study. J Clin Oncol. 2006;24(15):2352–8.PubMedCrossRef Dome JS, Cotton CA, Perlman EJ, et al. Treatment of anaplastic histology Wilms’ tumor: results from the fifth National Wilms’ Tumor Study. J Clin Oncol. 2006;24(15):2352–8.PubMedCrossRef
32.
go back to reference Ehrlich P, Chi YY, Chintagumpala MM, et al. Results of the first prospective multi-institutional treatment study in children with bilateral Wilms Tumor (AREN0534): a Report from the children’s Oncology Group. Ann Surg. 2017;266(3):470–8.PubMedCrossRef Ehrlich P, Chi YY, Chintagumpala MM, et al. Results of the first prospective multi-institutional treatment study in children with bilateral Wilms Tumor (AREN0534): a Report from the children’s Oncology Group. Ann Surg. 2017;266(3):470–8.PubMedCrossRef
33.
go back to reference Karam S, Gebreil A, Alksas A, Balaha HM, Khalil A, Ghazal M, Contractor S, El-Baz A. Insights into Personalized Care strategies for Wilms Tumor: a narrative literature review. Biomedicines. 2024;12(7):1455.PubMedPubMedCentralCrossRef Karam S, Gebreil A, Alksas A, Balaha HM, Khalil A, Ghazal M, Contractor S, El-Baz A. Insights into Personalized Care strategies for Wilms Tumor: a narrative literature review. Biomedicines. 2024;12(7):1455.PubMedPubMedCentralCrossRef
34.
go back to reference Hong B, Dong R. Research advances in the targeted therapy and immunotherapy of Wilms tumor: a narrative review. Translational cancer Res. 2021;10(3):1559–67.CrossRef Hong B, Dong R. Research advances in the targeted therapy and immunotherapy of Wilms tumor: a narrative review. Translational cancer Res. 2021;10(3):1559–67.CrossRef
35.
go back to reference Hont AB, Dumont B, Sutton KS, Anderson J, Kentsis A, Drost J, Hong AL, Verschuur A. The tumor microenvironment and immune targeting therapy in pediatric renal tumors. Pediatr Blood Cancer. 2023;70(Suppl 2):e30110.PubMedCrossRef Hont AB, Dumont B, Sutton KS, Anderson J, Kentsis A, Drost J, Hong AL, Verschuur A. The tumor microenvironment and immune targeting therapy in pediatric renal tumors. Pediatr Blood Cancer. 2023;70(Suppl 2):e30110.PubMedCrossRef
36.
go back to reference Majzner RG, Theruvath JL, Nellan A, et al. CAR T cells target-ing B7-H3, a pan-cancer antigen, demonstrate potent preclini-cal activity against pediatric solid tumors and brain tumors. ClinCancer Res. 2019;25(8):2560–74. Majzner RG, Theruvath JL, Nellan A, et al. CAR T cells target-ing B7-H3, a pan-cancer antigen, demonstrate potent preclini-cal activity against pediatric solid tumors and brain tumors. ClinCancer Res. 2019;25(8):2560–74.
37.
go back to reference Hont AB, Cruz CR, Ulrey R, O’Brien B, Stanojevic M, Datar A, Albihani S, Saunders D, Hanajiri R, Panchapakesan K, Darko S, Banerjee P, Fortiz MF, Hoq F, Lang H, Wang Y, Hanley PJ, Dome JS, Bollard CM, Meany HJ. Immunotherapy of Relapsed and Refractory Solid Tumors with Ex vivo expanded Multi-tumor Associated Antigen Specific cytotoxic T lymphocytes: a phase I study. J Clin Oncology: Official J Am Soc Clin Oncol. 2019;37(26):2349–59.CrossRef Hont AB, Cruz CR, Ulrey R, O’Brien B, Stanojevic M, Datar A, Albihani S, Saunders D, Hanajiri R, Panchapakesan K, Darko S, Banerjee P, Fortiz MF, Hoq F, Lang H, Wang Y, Hanley PJ, Dome JS, Bollard CM, Meany HJ. Immunotherapy of Relapsed and Refractory Solid Tumors with Ex vivo expanded Multi-tumor Associated Antigen Specific cytotoxic T lymphocytes: a phase I study. J Clin Oncology: Official J Am Soc Clin Oncol. 2019;37(26):2349–59.CrossRef
38.
go back to reference Alfaifi J. miRNAs role in Wilms tumor pathogenesis: signaling pathways interplay. Pathol Res Pract. 2024;256:155254.PubMedCrossRef Alfaifi J. miRNAs role in Wilms tumor pathogenesis: signaling pathways interplay. Pathol Res Pract. 2024;256:155254.PubMedCrossRef
39.
go back to reference Avčin SL, Črepinšek K, Jenko, Bizjan B, Šket R, Kovač J, Vrhovšek B, Blazina J, Blatnik O, Kordič R, Kitanovski L, Jazbec J, Debeljak M, Tesovnik T. Integrative transcriptomic profiling of the Wilms Tumor. Cancers. 2023;15(15):3846.PubMedPubMedCentralCrossRef Avčin SL, Črepinšek K, Jenko, Bizjan B, Šket R, Kovač J, Vrhovšek B, Blazina J, Blatnik O, Kordič R, Kitanovski L, Jazbec J, Debeljak M, Tesovnik T. Integrative transcriptomic profiling of the Wilms Tumor. Cancers. 2023;15(15):3846.PubMedPubMedCentralCrossRef
41.
go back to reference Shrestha AL, Shrestha P, Mishra A, Pandit A. A cystic non-wilms renal tumor in a Nepalese boy: a rare case of clear cell sarcoma. Int J Surg case Rep. 2023;109:108582.PubMedPubMedCentralCrossRef Shrestha AL, Shrestha P, Mishra A, Pandit A. A cystic non-wilms renal tumor in a Nepalese boy: a rare case of clear cell sarcoma. Int J Surg case Rep. 2023;109:108582.PubMedPubMedCentralCrossRef
42.
go back to reference Fiore M, Taddia A, Indio V, Bertuccio SN, Messelodi D, Serravalle S, Bandini J, Spreafico F, Perotti D, Collini P, Di Cataldo A, Pasquinelli G, Chiarini F, Fois M, Melchionda F, Pession A, Astolfi A. Molecular signature of Biological aggressiveness in Clear Cell Sarcoma of the kidney (CCSK). Int J Mol Sci. 2023;24(4):3743.PubMedPubMedCentralCrossRef Fiore M, Taddia A, Indio V, Bertuccio SN, Messelodi D, Serravalle S, Bandini J, Spreafico F, Perotti D, Collini P, Di Cataldo A, Pasquinelli G, Chiarini F, Fois M, Melchionda F, Pession A, Astolfi A. Molecular signature of Biological aggressiveness in Clear Cell Sarcoma of the kidney (CCSK). Int J Mol Sci. 2023;24(4):3743.PubMedPubMedCentralCrossRef
43.
go back to reference Aviral G, Sarvesh CM, Sushila J, Ansari MS. Clear cell sarcoma of kidney: a mimicker of Wilms’ tumor. J Cancer Res Ther. 2023;19(5):1468–70.PubMedCrossRef Aviral G, Sarvesh CM, Sushila J, Ansari MS. Clear cell sarcoma of kidney: a mimicker of Wilms’ tumor. J Cancer Res Ther. 2023;19(5):1468–70.PubMedCrossRef
44.
go back to reference Zhang A, Yuan X, Jiang S, Xu D, Huang C, Tang JY, Gao Y. Outcomes of children with clear cell sarcoma of kidney following NWTS strategies in Shanghai China (2003–2021). PLoS ONE. 2024;19(7):e0306863.PubMedPubMedCentralCrossRef Zhang A, Yuan X, Jiang S, Xu D, Huang C, Tang JY, Gao Y. Outcomes of children with clear cell sarcoma of kidney following NWTS strategies in Shanghai China (2003–2021). PLoS ONE. 2024;19(7):e0306863.PubMedPubMedCentralCrossRef
45.
go back to reference Laasri K, Bahlouli N, Chait F, Isfaoun Z, Hessissen L, Rouas L, Lamalmi N, Allali N, El Haddad S. Chat, L. A rare case of renal tumor in children: clear cell sarcoma with an unusual presentation. Radiol case Rep. 2023;18(11):3865–71.PubMedPubMedCentralCrossRef Laasri K, Bahlouli N, Chait F, Isfaoun Z, Hessissen L, Rouas L, Lamalmi N, Allali N, El Haddad S. Chat, L. A rare case of renal tumor in children: clear cell sarcoma with an unusual presentation. Radiol case Rep. 2023;18(11):3865–71.PubMedPubMedCentralCrossRef
46.
go back to reference Zhang M, Yao X, Guan X, Jia C, Zhang R, Wang H, Guo Y, Ni X, Yu Y, He L. Clinical relevance of BCOR internal tandem duplication and TP53 aberration in clear cell sarcoma of the kidney. Hum Pathol. 2023;134:45–55.PubMedCrossRef Zhang M, Yao X, Guan X, Jia C, Zhang R, Wang H, Guo Y, Ni X, Yu Y, He L. Clinical relevance of BCOR internal tandem duplication and TP53 aberration in clear cell sarcoma of the kidney. Hum Pathol. 2023;134:45–55.PubMedCrossRef
47.
48.
go back to reference Argani P, Perlman EJ, Breslow NE, et al. Clear cell sarcoma of the kidney: a review of 351 cases from the National Wilms Tumor Study Group Pathology Center. Am J Surg Pathol. 2000;24(1):4.PubMedCrossRef Argani P, Perlman EJ, Breslow NE, et al. Clear cell sarcoma of the kidney: a review of 351 cases from the National Wilms Tumor Study Group Pathology Center. Am J Surg Pathol. 2000;24(1):4.PubMedCrossRef
49.
go back to reference Argani P, Pawel B, Szabo S, Reyes-Múgica M, Timmons C, Antonescu CR. Diffuse strong BCOR immunoreactivity is a sensitive and specific marker for clear cell sarcoma of the kidney (CCSK) in pediatric renal neoplasia. Am J Surg Pathol. 2018;42(8):1128–31.PubMedPubMedCentralCrossRef Argani P, Pawel B, Szabo S, Reyes-Múgica M, Timmons C, Antonescu CR. Diffuse strong BCOR immunoreactivity is a sensitive and specific marker for clear cell sarcoma of the kidney (CCSK) in pediatric renal neoplasia. Am J Surg Pathol. 2018;42(8):1128–31.PubMedPubMedCentralCrossRef
50.
go back to reference Gooskens SL, Graf N, Furtwängler R, Spreafico F, Bergeron C, Ramírez-Villar GL, Godzinski J, Rübe C, Janssens GO, Vujanic GM, Leuschner I, Coulomb-L’Hermine A, Smets AM, de Camargo B, Stoneham S, van Tinteren H, Pritchard-Jones K, van den Heuvel-Eibrink MM. International Society of Paediatric Oncology–Renal Tumour Study Group (SIOP–RTSG). Position paper: Rationale for the treatment of children with CCSK in the UMBRELLA SIOP-RTSG 2016 protocol. Nat Rev Urol. 2028;15(5):309–19.CrossRef Gooskens SL, Graf N, Furtwängler R, Spreafico F, Bergeron C, Ramírez-Villar GL, Godzinski J, Rübe C, Janssens GO, Vujanic GM, Leuschner I, Coulomb-L’Hermine A, Smets AM, de Camargo B, Stoneham S, van Tinteren H, Pritchard-Jones K, van den Heuvel-Eibrink MM. International Society of Paediatric Oncology–Renal Tumour Study Group (SIOP–RTSG). Position paper: Rationale for the treatment of children with CCSK in the UMBRELLA SIOP-RTSG 2016 protocol. Nat Rev Urol. 2028;15(5):309–19.CrossRef
51.
go back to reference He M, Cai J, Zhu K, Gu W, Li M, Xiong J, Guan Z, Wang J, Shu Q. Renal cell carcinoma in children and adolescents: single-center experience and literature review. Medicine. 2021;100(2):e23717.PubMedPubMedCentralCrossRef He M, Cai J, Zhu K, Gu W, Li M, Xiong J, Guan Z, Wang J, Shu Q. Renal cell carcinoma in children and adolescents: single-center experience and literature review. Medicine. 2021;100(2):e23717.PubMedPubMedCentralCrossRef
52.
go back to reference Chaabouni A, Samet A, Fourati M, Mejdoub B, Kammoun O, Mseddi MA, Hadjslimene M. Renal cell carcinoma in children, report of a new case. Urol case Rep. 2021;39:101813.PubMedPubMedCentralCrossRef Chaabouni A, Samet A, Fourati M, Mejdoub B, Kammoun O, Mseddi MA, Hadjslimene M. Renal cell carcinoma in children, report of a new case. Urol case Rep. 2021;39:101813.PubMedPubMedCentralCrossRef
53.
go back to reference Khondker A, Kwong JCC, Chua ME, Kim JK, Chan JYH, Zappitelli M, Brzezinski J, Cost NG, Rickard M, Lorenzo AJ. Nephron-sparing surgery for renal cell carcinoma in children and young adults: a systematic review. Urol Oncol. 2023;41(3):137–44.PubMedCrossRef Khondker A, Kwong JCC, Chua ME, Kim JK, Chan JYH, Zappitelli M, Brzezinski J, Cost NG, Rickard M, Lorenzo AJ. Nephron-sparing surgery for renal cell carcinoma in children and young adults: a systematic review. Urol Oncol. 2023;41(3):137–44.PubMedCrossRef
54.
go back to reference Denize T, Massa S, Valent A, Militti L, Bertolotti A, Barisella M, Rioux-Leclercq N, Malouf GG, Spreafico F, Verschuur A, van der Beek J, Tytgat L, van den Heuvel-Eibrink MM, Vujanic G, Collini P, Coulomb A. Renal cell carcinoma in children and adolescents: a retrospective study of a french-italian series of 93 cases. Histopathology. 2022;80(6):928–45.PubMedCrossRef Denize T, Massa S, Valent A, Militti L, Bertolotti A, Barisella M, Rioux-Leclercq N, Malouf GG, Spreafico F, Verschuur A, van der Beek J, Tytgat L, van den Heuvel-Eibrink MM, Vujanic G, Collini P, Coulomb A. Renal cell carcinoma in children and adolescents: a retrospective study of a french-italian series of 93 cases. Histopathology. 2022;80(6):928–45.PubMedCrossRef
55.
go back to reference van der Beek JN, Hol JA, Coulomb-l’Hermine A, Graf N, van Tinteren H, Pritchard-Jones K, Houwing ME, de Krijger RR, Vujanic GM, Dzhuma K, Schenk JP, Littooij AS, Ramírez-Villar GL, Murphy D, Ray S, Al-Saadi R, Gessler M, Godzinski J, Ruebe C, Collini P, van den Heuvel-Eibrink MM. Characteristics and outcome of pediatric renal cell carcinoma patients registered in the International Society of Pediatric Oncology (SIOP) 93– 01, 2001 and UK-IMPORT database: a report of the SIOP-Renal Tumor Study Group. Int J Cancer. 2021;148(11):2724–35.PubMedPubMedCentralCrossRef van der Beek JN, Hol JA, Coulomb-l’Hermine A, Graf N, van Tinteren H, Pritchard-Jones K, Houwing ME, de Krijger RR, Vujanic GM, Dzhuma K, Schenk JP, Littooij AS, Ramírez-Villar GL, Murphy D, Ray S, Al-Saadi R, Gessler M, Godzinski J, Ruebe C, Collini P, van den Heuvel-Eibrink MM. Characteristics and outcome of pediatric renal cell carcinoma patients registered in the International Society of Pediatric Oncology (SIOP) 93– 01, 2001 and UK-IMPORT database: a report of the SIOP-Renal Tumor Study Group. Int J Cancer. 2021;148(11):2724–35.PubMedPubMedCentralCrossRef
56.
go back to reference Cajaiba MM, Dyer LM, Geller JI, Jennings LJ, George D, Kirschmann D, Rohan SM, Cost NG, Khanna G, Mullen EA, Dome JS, Fernandez CV, Perlman EJ. The classification of pediatric and young adult renal cell carcinomas registered on the children’s oncology group (COG) protocol AREN03B2 after focused genetic testing. Cancer. 2018;124(16):3381–9.PubMedCrossRef Cajaiba MM, Dyer LM, Geller JI, Jennings LJ, George D, Kirschmann D, Rohan SM, Cost NG, Khanna G, Mullen EA, Dome JS, Fernandez CV, Perlman EJ. The classification of pediatric and young adult renal cell carcinomas registered on the children’s oncology group (COG) protocol AREN03B2 after focused genetic testing. Cancer. 2018;124(16):3381–9.PubMedCrossRef
57.
go back to reference Rao Q, Chen JY, Wang JD, Ma HH, Zhou HB, Lu ZF, Zhou XJ. Renal cell carcinoma in children and young adults: clinicopathological, immunohistochemical, and VHL gene analysis of 46 cases with follow-up. Int J Surg Pathol. 2011;19(2):170–9.PubMedCrossRef Rao Q, Chen JY, Wang JD, Ma HH, Zhou HB, Lu ZF, Zhou XJ. Renal cell carcinoma in children and young adults: clinicopathological, immunohistochemical, and VHL gene analysis of 46 cases with follow-up. Int J Surg Pathol. 2011;19(2):170–9.PubMedCrossRef
58.
go back to reference Ray S, Jones R, Pritchard-Jones K, Dzhuma K, van den Heuvel-Eibrink M, Tytgat G, van der Beek J, Oades G, Murphy D. Pediatric and young adult renal cell carcinoma. Pediatr Blood Cancer. 2020;67(11):e28675.PubMedCrossRef Ray S, Jones R, Pritchard-Jones K, Dzhuma K, van den Heuvel-Eibrink M, Tytgat G, van der Beek J, Oades G, Murphy D. Pediatric and young adult renal cell carcinoma. Pediatr Blood Cancer. 2020;67(11):e28675.PubMedCrossRef
59.
go back to reference Brok J, Treger TD, Gooskens SL, van den Heuvel-Eibrink MM, Pritchard-Jones K. Biology and treatment of renal tumours in childhood. European journal of cancer (Oxford, England: 1990). 2026; 68, 179–195. Brok J, Treger TD, Gooskens SL, van den Heuvel-Eibrink MM, Pritchard-Jones K. Biology and treatment of renal tumours in childhood. European journal of cancer (Oxford, England: 1990). 2026; 68, 179–195.
60.
go back to reference Ljungberg B, Bensalah K, Canfield S, Dabestani S, Hofmann F, Hora M, Kuczyk MA, Lam T, Marconi L, Merseburger AS, Mulders P, Powles T, Staehler M, Volpe A, Bex A. EAU guidelines on renal cell carcinoma: 2014 update. European urology. 2015; 67(5): 913–924. Ljungberg B, Bensalah K, Canfield S, Dabestani S, Hofmann F, Hora M, Kuczyk MA, Lam T, Marconi L, Merseburger AS, Mulders P, Powles T, Staehler M, Volpe A, Bex A. EAU guidelines on renal cell carcinoma: 2014 update. European urology. 2015; 67(5): 913–924.
61.
go back to reference Zhanghuang C, Zhang Z, Zeng L, Yan B, Tang H, Wang J, Liu X, Wei G, He D. Clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney: a 7-year retrospective multi-center study. BMC Pediatr. 2022;22(1):591.PubMedPubMedCentralCrossRef Zhanghuang C, Zhang Z, Zeng L, Yan B, Tang H, Wang J, Liu X, Wei G, He D. Clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney: a 7-year retrospective multi-center study. BMC Pediatr. 2022;22(1):591.PubMedPubMedCentralCrossRef
62.
go back to reference Xie S, Fang Y, Yang Y, Liu L, Bai J, Lin S, Zhang B, Fang Y. Extracranial malignant rhabdoid tumors in children: high mortality even with the help of an aggressive clinical approach. Eur J Pediatrics. 2024;183(2):557–67.CrossRef Xie S, Fang Y, Yang Y, Liu L, Bai J, Lin S, Zhang B, Fang Y. Extracranial malignant rhabdoid tumors in children: high mortality even with the help of an aggressive clinical approach. Eur J Pediatrics. 2024;183(2):557–67.CrossRef
63.
go back to reference Schenk JP, Hötker A, Furtwängler R, Fuchs J, Warmann SW, Graf N. Bildgebung renaler Tumoren Im Kindesalter [Imaging of renal tumors in children]. Radiologe. 2021;61(7):619–28.PubMedCrossRef Schenk JP, Hötker A, Furtwängler R, Fuchs J, Warmann SW, Graf N. Bildgebung renaler Tumoren Im Kindesalter [Imaging of renal tumors in children]. Radiologe. 2021;61(7):619–28.PubMedCrossRef
64.
go back to reference Li J, Zhang W, Hu H, Zhang Y, Wang Y, Gu H. Huang, D. Case Analysis of 14 children with malignant rhabdoid tumor of the kidney. Cancer Manage Res. 2021;13:4865–72.CrossRef Li J, Zhang W, Hu H, Zhang Y, Wang Y, Gu H. Huang, D. Case Analysis of 14 children with malignant rhabdoid tumor of the kidney. Cancer Manage Res. 2021;13:4865–72.CrossRef
65.
go back to reference Qureshi SS, Bhagat M, Verma K, Yadav S, Prasad M, Vora T, Chinnaswamy G, Amin N, Smriti V, Baheti A, Laskar S, Khanna N, Ramadwar M, Shah S. Incidence, treatment, and outcomes of primary and recurrent Non-Wilms renal tumors in children: Report of 109 patients treated at a single institution. Journal of pediatric urology. 2020; 16(4):, 475.e1–475.e9. Qureshi SS, Bhagat M, Verma K, Yadav S, Prasad M, Vora T, Chinnaswamy G, Amin N, Smriti V, Baheti A, Laskar S, Khanna N, Ramadwar M, Shah S. Incidence, treatment, and outcomes of primary and recurrent Non-Wilms renal tumors in children: Report of 109 patients treated at a single institution. Journal of pediatric urology. 2020; 16(4):, 475.e1–475.e9.
66.
go back to reference van den Heuvel-Eibrink MM, van Tinteren H, Rehorst H, Coulombe A, Patte C, de Camargo B, et al. Malignant rhabdoid tumours of the kidney (MRTKs), registered on recent SIOP protocols from 1993 to 2005: a report of the SIOP renal tumour study group. Pediatr Blood Canc. 2011;56:733–7.CrossRef van den Heuvel-Eibrink MM, van Tinteren H, Rehorst H, Coulombe A, Patte C, de Camargo B, et al. Malignant rhabdoid tumours of the kidney (MRTKs), registered on recent SIOP protocols from 1993 to 2005: a report of the SIOP renal tumour study group. Pediatr Blood Canc. 2011;56:733–7.CrossRef
67.
go back to reference Tomlinson GE, Breslow NE, Dome J, Guthrie KA, Norkool P, Li S et al. Rhabdoid tumour of the kidney in the National Wilms’ Tumor Study: age at diagnosis as a prognostic factor. J Clin Oncol. 2005; 23: pp. 7641–7645. Tomlinson GE, Breslow NE, Dome J, Guthrie KA, Norkool P, Li S et al. Rhabdoid tumour of the kidney in the National Wilms’ Tumor Study: age at diagnosis as a prognostic factor. J Clin Oncol. 2005; 23: pp. 7641–7645.
68.
go back to reference Grosinger L, Salik I, Mehta B. Infantile congenital Mesoblastic Nephroma leading to multi-systemic end-organ disease. Cureus. 2022;14(10):e30513.PubMedPubMedCentral Grosinger L, Salik I, Mehta B. Infantile congenital Mesoblastic Nephroma leading to multi-systemic end-organ disease. Cureus. 2022;14(10):e30513.PubMedPubMedCentral
69.
go back to reference Rayner J, Vinycomb T, Wanaguru D, Jiwane A. Congenital mesoblastic nephroma: review of current management and outcomes in a single centre. ANZ J Surg. 2023;93(4):1008–11.PubMedCrossRef Rayner J, Vinycomb T, Wanaguru D, Jiwane A. Congenital mesoblastic nephroma: review of current management and outcomes in a single centre. ANZ J Surg. 2023;93(4):1008–11.PubMedCrossRef
70.
go back to reference Che M, Yang F, Huang H, Zhang H, Han C, Sun N. Prenatal diagnosis of fetal congenital mesoblastic nephroma by ultrasonography combined with MR imaging: a case report and literature review. Medicine. 2021, 100(3), e24034. Che M, Yang F, Huang H, Zhang H, Han C, Sun N. Prenatal diagnosis of fetal congenital mesoblastic nephroma by ultrasonography combined with MR imaging: a case report and literature review. Medicine. 2021, 100(3), e24034.
71.
go back to reference Simkhada A, Paudel R, Sharma N. Congenital Mesoblastic Nephroma: a Case Report. JNMA. 2023;61(259):259–62. Simkhada A, Paudel R, Sharma N. Congenital Mesoblastic Nephroma: a Case Report. JNMA. 2023;61(259):259–62.
72.
go back to reference Chung EM, Graeber AR, Conran RM. Renal tumors of Childhood: radiologic-pathologic correlation part 1. The 1st Decade: from the Radiologic Pathology archives. Radiographics: Rev Publication Radiological Soc North Am Inc. 2016;36(2):499–522.CrossRef Chung EM, Graeber AR, Conran RM. Renal tumors of Childhood: radiologic-pathologic correlation part 1. The 1st Decade: from the Radiologic Pathology archives. Radiographics: Rev Publication Radiological Soc North Am Inc. 2016;36(2):499–522.CrossRef
73.
go back to reference Ooms AHAG, Vujanić GM, D’Hooghe E, Collini P, L’Herminé-Coulomb A, Vokuhl C, Graf N, Heuvel-Eibrink MMVD, de Krijger RR. Renal tumors of Childhood-A histopathologic pattern-based Diagnostic Approach. Cancers. 2020;12(3):729.PubMedPubMedCentralCrossRef Ooms AHAG, Vujanić GM, D’Hooghe E, Collini P, L’Herminé-Coulomb A, Vokuhl C, Graf N, Heuvel-Eibrink MMVD, de Krijger RR. Renal tumors of Childhood-A histopathologic pattern-based Diagnostic Approach. Cancers. 2020;12(3):729.PubMedPubMedCentralCrossRef
74.
go back to reference Santos LG, Carvalho JdeS, Reis MA, Sales RL. Cellular congenital mesoblastic nephroma: case report. Jornal brasileiro de nefrologia. 2011;33(1):109–12.PubMedCrossRef Santos LG, Carvalho JdeS, Reis MA, Sales RL. Cellular congenital mesoblastic nephroma: case report. Jornal brasileiro de nefrologia. 2011;33(1):109–12.PubMedCrossRef
75.
go back to reference Ahmed HU, Arya M, Levitt G, Duffy PG, Mushtaq I, Sebire NJ, Part I. Primary malignant non-wilms’ renal tumours in children. Lancet Oncol. 2007;8(8):730–7.PubMedCrossRef Ahmed HU, Arya M, Levitt G, Duffy PG, Mushtaq I, Sebire NJ, Part I. Primary malignant non-wilms’ renal tumours in children. Lancet Oncol. 2007;8(8):730–7.PubMedCrossRef
Metadata
Title
Paediatric Renal Tumors: A State-of-the-Art Review
Authors
Cecilia Salzillo
Gerardo Cazzato
Gabriella Serio
Andrea Marzullo
Publication date
07-02-2025

Elevate your expertise in aplastic anemia (Link opens in a new window)

Transform the way you care for your patients with aplastic anemia with our 3-module series using real-world case studies and expert insights. Discover why early diagnosis matters, explore the benefits and risks of current treatments, and develop tailored approaches for complex cases. 

Supported by:
  • Pfizer
Developed by: Springer Healthcare IME
Learn more
SPONSORED

Recent advances in the use of CAR T-cell therapies in relapsed/refractory diffuse large B-cell lymphoma and follicular lymphoma

In this webinar, Professor Martin Dreyling and an esteemed international panel of CAR T-cell therapy experts discuss the latest data on the safety, efficacy, and clinical impact of CAR T-cell therapies in the treatment of r/r DLBCL and r/r FL.

Please note, this webinar is not intended for healthcare professionals based in the US and UK.

Sponsored by:
  • Novartis Pharma AG
Chaired by: Prof. Martin Dreyling
Developed by: Springer Healthcare
Watch now
Video