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Pathology

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Wilms’ Tumor
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Abstract

Wilms’ tumor (WT) is the most prevalent renal tumor of childhood. It is a malignant embryonal neoplasm taking its origin from embryonic renal precursor cells and showing divergent differentiation. WT can present monofocal or multifocal lesion(s), in unilateral or bilateral kidneys. The characteristic histological appearance is that of a triphasic tumor, comprising of blastemal, epithelial, and mesenchymal components. Not uncommonly, biphasic or monophasic tumors can also be seen, which may cause diagnostic dilemma. Anaplastic features can be identified in any of the three components and are important predictors for chemotherapy (ChT) resistance and poor prognosis. Neoadjuvant ChT is a common treatment modality for WT; hence, post-ChT specimens are thoroughly examined and evaluated to assess the response to therapy and presence of residual tumor.

Learning objectives of this chapter include the knowledge of the histomorphologic spectrum of WT, its morphologic mimics, and how to differentiate them. Handling of resection specimens and evaluation of post-ChT specimens for staging and risk stratification are also discussed.

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References

  1. Sebire NJ, Vujanic GM. Paediatric renal tumours: recent developments, new entities and pathological features. Histopathology. 2009;54:516–28. https://doi.org/10.1111/j.1365-2559.2008.03110.x.

    Article  PubMed  Google Scholar 

  2. Varan A. Wilms’ tumor in children: an overview. Clin Pract. 2008;108:83–90. https://doi.org/10.1159/000113012.

    Article  Google Scholar 

  3. Chu A, Heck JE, Ribeiro KB, Brennan P, Boffetta P, Buffler P, et al. Wilms’ tumour: a systematic review of risk factors and meta-analysis. Paediatr Perinat Epidemiol. 2010;24:449–69. https://doi.org/10.1111/j.1365-3016.2010.01133.x.

    Article  PubMed  Google Scholar 

  4. Davidoff AM. Wilms tumor. Adv Pediatr Infect Dis. 2012;59:247–67. https://doi.org/10.1016/j.yapd.2012.04.001.

    Article  Google Scholar 

  5. Argani P, Bruder E, Dehner L, Vujanic GM. Nephroblastic and cystic tumors occuring mainly in children. In: Moch H, Humphrey PA, Ulbright TM, Reuter VE, editors. WHO classification of tumours of the urinary system and male genital organs. 4th ed. Lyon: IARC; 2016. p. 48–53.

    Google Scholar 

  6. Vujanić GM, Sandstedt B. The pathology of Wilms’ tumour (nephroblastoma): The International Society of Paediatric Oncology approach. J Clin Pathol. 2010;63:102–9. https://doi.org/10.1136/jcp.2009.064600.

    Article  PubMed  Google Scholar 

  7. Ramchandra C, Attili VSS, Dadhich HP, Kumari A, Appaji L, Giri GV, et al. Extrarenal Wilms’ tumor: a report of two cases and review of literature. J Indian Assoc Pediatr Surg. 2007;12:145–7.

    Article  Google Scholar 

  8. Thakkar NC, Sarin YK. Extra-renal Wilms’ tumor: a rare diagnosis. APSP J Case Rep. 2015;6:17.

    PubMed  PubMed Central  Google Scholar 

  9. Al-Hussain T, Ali A, Akhtar M. Wilms tumor: an update. Adv Anat Pathol. 2014;21:166–73. https://doi.org/10.1097/PAP.0000000000000017.

    Article  PubMed  Google Scholar 

  10. Hohenstein P, Pritchard-Jones K, Charlton J. The yin and yang of kidney development and Wilms’ tumors. Genes Dev. 2015;29:467–82.

    Article  CAS  Google Scholar 

  11. Perlman EJ. Pediatric renal tumors: practical updates for the pathologist. Pediatr Dev Pathol. 2005;8:320–38. https://doi.org/10.1007/s10024-005-1156-7.

    Article  PubMed  Google Scholar 

  12. Vujanić GM, Sandstedt B, Harms D, Leuschner I, Kelsey A, dE Kraker J. Revised International Society of Paediatric Oncology (SIOP) working classification of renal tumors of childhood. Med Pediatr Oncol. 2002;38:79–82. https://doi.org/10.1002/mpo.1276.

    Article  PubMed  Google Scholar 

  13. 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;47:260–7. https://doi.org/10.1002/pbc.20891.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Popov SD, Sebire NJ, Vujanic GM. Wilms’ tumour – histology and differential diagnosis. In: van den Heuvel-Eibrink MM, editor. Wilms tumor. Brisbane: Codon Publications; 2016.

    Google Scholar 

  15. Agrani P, Beckwith JB. Renal neoplasms of childhood. In: Mills ES, editor. Sternberg’s diagnostic surgical pathology. 6th ed. Philadelphia: Wolter Kluwer Health; 2015. p. 4194–259.

    Google Scholar 

  16. Vujanić GM, Schiavo Lena M, Sebire NJ. Botryoid Wilms tumor: a non-existent “entity” causing diagnostic and staging difficulties. Virchows Arch. 2019;474:227–34. https://doi.org/10.1007/s00428-018-2500-4.

    Article  PubMed  Google Scholar 

  17. Ghamdi DAL, Bakshi N, Akhtar M. Teratoid wilms tumor: report of three cases and review of the literature. Turk Patoloji Derg. 2019;35:61–8. https://doi.org/10.5146/tjpath.2016.01363.

    Article  PubMed  Google Scholar 

  18. Beckwith JB, Zuppan CE, Browning NG, Moksness J, Breslow NE. Histological analysis of aggressiveness and responsiveness in Wilms’ tumor. Med Pediatr Oncol. 1996;27:422–8. https://doi.org/10.1002/(SICI)1096-911X(199611)27:5<422::AID-MPO6>3.0.CO;2-O.

    Article  CAS  PubMed  Google Scholar 

  19. Beckwith JB. National Wilms tumor study: an update for pathologists. Pediatr Dev Pathol. 1998;1:79–84. https://doi.org/10.1007/s100249900010.

    Article  CAS  PubMed  Google Scholar 

  20. Daw NC, Chi YY, Kim Y, Mullen EA, Kalapurakal JA, Tian J, et al. Treatment of stage I anaplastic Wilms’ tumour: a report from the Children’s Oncology Group AREN0321 study. Eur J Cancer. 2019;118:58–66. https://doi.org/10.1016/j.ejca.2019.05.033.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Vujanic GM, Harms D, Sandstedt B, Weirich A, de Kraker J, Delemarre JFM. New definitions of focal and diffuse anaplasia in Wilms tumor: the International Society of Paediatric Oncology (SIOP) experience. Med Pediatr Oncol. 1999;32:317–23. https://doi.org/10.1002/(sici)1096-911x(199905)32:5<317::aid-mpo1>3.0.co;2-f.

    Article  CAS  PubMed  Google Scholar 

  22. Faria P, Beckwith JB. A new definition of focal anaplasia in Wilms tumor identifies cases with good outcome: a report from the National Wilms Tumor Study. Mod Pathol. 1993;6:3.

    Google Scholar 

  23. Rudzinki E, Perlman E, Kim G, Sebire N. College of American Pathologists Protocol for examination of biopsy specimens from patients with Wilms and other pediatric renal tumors. Version: Wilms tumor biopsy 4.0.0.0. 2019. Available from https://www.cap.org/protocols-and-guidelines/cancer-reporting-tools/cancer-protocol-templates. Accessed 31 March 2020.

  24. Alvarez-Silvan AM, Carrasco FC, Cuevas CP, Becerra EA, Anguita ML, Caro AM, et al. Preservation of anaplastic features in Wilms’ tumors after preoperative chemotherapy. Med Pediatr Oncol. 1989;17:131–3. https://doi.org/10.1002/mpo.2950170211.

    Article  CAS  PubMed  Google Scholar 

  25. Hill NA, Shear TD, Liu T, Billups CA, Singh PK, Dome JS. Clinical and biologic significance of nuclear unrest in Wilms tumor. Cancer. 2003;97:2318–26. https://doi.org/10.1002/cncr.11325.

    Article  PubMed  Google Scholar 

  26. Salama A, Kamel A. Evaluation of nuclear unrest and p53 immunostaining in Wilms’ tumor. J Egypt Natl Canc Inst. 2011;23:31–9. https://doi.org/10.1016/j.jnci.2011.07.005.

    Article  PubMed  Google Scholar 

  27. Zuppan CW, Beckwith JB, Luckey DW. Anaplasia in unilateral Wilms’ tumor: a report from the National Wilms’ Tumor Study Pathology Center. Hum Pathol. 1988;19:1199–209. https://doi.org/10.1016/s0046-8177(88)80152-7.

    Article  CAS  PubMed  Google Scholar 

  28. Guarda LA, Ayala AG, Jaffe N, Sutow WW, Bracken RB. Chemotherapy-induced histologic changes in Wilms’ tumors. Pediatr Pathol. 1984;2:197–206. https://doi.org/10.3109/15513818409025887.

    Article  CAS  PubMed  Google Scholar 

  29. Taskinen S, Lohi J, Koskenvuo M, Taskinen M. Evaluation of effect of preoperative chemotherapy on Wilms’ tumor histopathology. J Pediatr Surg. 2017;53:1611–4. https://doi.org/10.1016/j.jpedsurg.2017.10.002.

  30. Reinhard H, Semler O, Bürger D, Bode U, Flentje M, Göbel U, et al. Results of the SIOP 93-01/GPOH trial and study for the treatment of patients with unilateral nonmetastatic Wilms Tumor. Klin Padiatr. 2004;216:132–40. https://doi.org/10.1055/s-2004-822625.

    Article  CAS  PubMed  Google Scholar 

  31. Provenzi VO, Rosa RF, Rosa RC, Roehe AV, dos Santos PP, Faulhaber FR, et al. Tamanho tumoral e prognóstico em pacientes portadores de tumor de Wilms [Tumor size and prognosis in patients with Wilms tumor]. Rev Paul Pediatr. 2015;33:82–7. https://doi.org/10.1016/j.rpped.2014.05.003.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Vujanić GM, Gessler M, Ooms AHAG, Collini P, Coulomb Hermine A, D’Hooghe E, et al. The UMBRELLA SIOP–RTSG 2016 Wilms tumour pathology and molecular biology protocol. Nat Rev Urol. 2018(15):693–701. https://doi.org/10.1038/s41585-018-0100-3.

  33. Fernández-Pineda I, Cabello R, García-Cantón JA, Pérez-Bertolez S, Tuduri Í, Ramírez G, et al. Fine-needle aspiration cytopathology in the diagnosis of Wilms tumor. Clin Transl Oncol. 2011;13:809–11. https://doi.org/10.1007/s12094-011-0738-2.

    Article  PubMed  Google Scholar 

  34. Irtan S, Jitlal M, Bate J, Powis M, Vujanic G, Kelsey A, et al. Risk factors for local recurrence in Wilms tumour and the potential influence of biopsy - the United Kingdom experience. Eur J Cancer. 2015;51:225–32. https://doi.org/10.1016/j.ejca.2014.10.026.

    Article  CAS  PubMed  Google Scholar 

  35. Kurian JJ, Nongpiur KR, Jehangir S. Use of pretherapy core biopsy in the diagnosis of pediatric renal tumors. J Indian Assoc Pediatr Surg. 2018;23:66–9. https://doi.org/10.4103/jiaps.JIAPS_245_16.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Shet T, Viswanathan S. The cytological diagnosis of paediatric renal tumours. J Clin Pathol. 2009;62:961–9. https://doi.org/10.1136/jcp.2009.064659.

    Article  CAS  PubMed  Google Scholar 

  37. Verdeguer A, Castel V, Torres V, Olagüe R, Ferris J, Esquembre C, et al. Fine-needle aspiration biopsy in children: experience in 70 cases. Med Pediatr Oncol. 1988;16:98–100. https://doi.org/10.1002/mpo.2950160206.

    Article  CAS  PubMed  Google Scholar 

  38. Truong LD, Shen SS. Immunohistochemical diagnosis of renal neoplasms. Arch Pathol Lab Med. 2011;135:92–109. https://doi.org/10.1043/2010-0478-RAR.1.

    Article  PubMed  Google Scholar 

  39. Shen SS, Truong LD, Scarpelli M, Lopez-Beltran A. Role of immunohistochemistry in diagnosing renal neoplasms when is it really useful? Arch Pathol Lab Med. 2012;136:410–7. https://doi.org/10.5858/arpa.2011-0472-RA.

    Article  PubMed  Google Scholar 

  40. Chen BF, Tzen CY, Liang DC, Liu HC, Huang YW, Fan CC. Immunohistochemical expression of Wilms’ tumor 1 protein in nephroblastoma. J Chin Med Assoc. 2004;67:506–10.

    PubMed  Google Scholar 

  41. Geethamani V, Kusuma V, Gowda KMS, Saini ML. Adult Wilms’ tumour: a case report with review of literature. Diagn Pathol. 2006;1:46. https://doi.org/10.1186/1746-1596-1-46.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Fan R. Primary renal neuroblastoma-a clinical pathologic study of 8 cases. Am J Surg Pathol. 2012;36:94–100. https://doi.org/10.1097/PAS.0b013e318233083b.

    Article  PubMed  Google Scholar 

  43. Bartholow T, Parwani A. Renal primitive neuroectodermal tumors. Arch Pathol Lab Med. 2012;136:686–90. https://doi.org/10.5858/arpa.2011-0104-RS.

    Article  PubMed  Google Scholar 

  44. Hoot AC, Russo P, Judkins AR, Perlman EJ, Biegel JA. Immunohistochemical analysis of hSNF5/INI1 distinguishes renal and extra-renal malignant rhabdoid tumors from other pediatric soft tissue tumors. Am J Surg Pathol. 2004;28:1485–91. https://doi.org/10.1097/01.pas.0000141390.14548.34.

    Article  PubMed  Google Scholar 

  45. Bulbul A, Fahy BN, Xiu J, Rashad S, Mustafa A, Husain H, et al. Desmoplastic small round blue cell tumor: a review of treatment and potential therapeutic genomic alterations. Sarcoma. 2017;2017:1278268. https://doi.org/10.1155/2017/1278268.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Yellala A, Jani PM, Sandhu A, Patibandla NSK, Greenberg L, Schiffman S, et al. Primary renal synovial sarcoma – a diagnostic dilemma. JCSO. 2018;16:e202–5. https://doi.org/10.12788/jcso.0426.

    Article  Google Scholar 

  47. Kinney SN, Eble JN, Hes O, Williamson SR, Grignon DJ, Wang M, et al. Metanephric adenoma: the utility of immunohistochemical and cytogenetic analyses in differential diagnosis, including solid variant papillary renal cell carcinoma and epithelial-predominant nephroblastoma. Mod Pathol. 2015;28:1236–48. https://doi.org/10.1038/modpathol.2015.81.

    Article  CAS  PubMed  Google Scholar 

  48. Perlman EJ. Pediatric renal cell carcinoma. Surg Pathol Clin. 2010;3:641–51. https://doi.org/10.1016/j.path.2010.06.011.

    Article  PubMed  PubMed Central  Google Scholar 

  49. Charles AK, Vujanic GM, Berry PJ. Renal tumors of childhood. Histopathology. 1998;32:293–309. https://doi.org/10.1046/j.1365-2559.1998.00344.x.

    Article  CAS  PubMed  Google Scholar 

  50. Kurian JJ, Jehangir S, Korula A. Multiloculated cystic renal tumors of childhood: Has the final word been spoken. J Indian Assoc Pediatr Surg. 2018;23:22–6. https://doi.org/10.4103/jiaps.JIAPS_224_16.

    Article  PubMed  PubMed Central  Google Scholar 

  51. Dowerah S, Borgohain M. Cystic partially differentiated nephroblastoma: a rare case report. Ann Path Lab Med. 2015;2:155–8.

    Google Scholar 

  52. Qualman SJ, Bowen J, Amin MB, Srigley JR, Grundy PE, Perlman EJ. Protocol for the examination of specimens from patients with Wilms tumor (nephroblastoma) or other renal tumors of childhood. Arch Pathol Lab Med. 2003;127:1280–9. https://doi.org/10.1043/1543-2165(2003)127%3C1280:PFTEOS%3E2.0.CO;2.

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  54. Beckwith JB. Precursor lesions of Wilms tumor: clinical and biological implications. Med Pediatr Oncol. 1993;21:158–68. https://doi.org/10.1002/mpo.2950210303.

    Article  CAS  PubMed  Google Scholar 

  55. Caiulo VA, Latini G, Cataldi L, De Felice C. Nephrogenic rests: their frequency and their fate. J Pediatr Hematol Oncol. 2007;29:361–3. https://doi.org/10.1097/MPH.0b013e3180601058.

    Article  PubMed  Google Scholar 

  56. Mishra K, Mathur M, Logani KB, Kakkar N, Krishna A. Precursor lesions of Wilms’ tumor in Indian children: a multiinstitutional study. Cancer. 1998;83:2228–32.

    Article  CAS  Google Scholar 

  57. Stabouli S, Printza N, Dotis J, Matis A, Koliouskas D, Gombakis N, et al. Perilobar nephroblastomatosis: natural history and management. Case Rep Pediatr. 2014;2014:1–7. https://doi.org/10.1155/2014/756819.

    Article  Google Scholar 

  58. Wu Y, Zhu X, Wang X, Wang H, Cao X, Wang J. Extrarenal nephroblastomatosis in children: a report of two cases. BMC Pediatr. 2014;14:255. https://doi.org/10.1186/1471-2431-14-255.

    Article  PubMed  PubMed Central  Google Scholar 

  59. Hennigar RA, O'Shea PA, Grattan-Smith JD. Clinicopathologic features of nephrogenic rests and nephroblastomatosis. Adv Anat Pathol. 2001;8:276–89. https://doi.org/10.1097/00125480-200109000-00005.

    Article  CAS  PubMed  Google Scholar 

  60. Gao B, Nzekwu E, Cook AJ, Spaner SJ. Case report: diffuse hyperplastic perilobar nephroblastomatosis complicated by a unilateral Wilms tumour: diagnosis, treatment and follow-up. BMC Res Notes. 2018;11:396. https://doi.org/10.1186/s13104-018-3502-7.

    Article  PubMed  PubMed Central  Google Scholar 

  61. Vuononvirta R, Sebire NJ, Dallosso AR, Reis-Filho JS, Williams RD, Mackay A, et al. Perilobar nephrogenic rests are nonobligate molecular genetic precursor lesions of insulin-like growth factor-ii-associated Wilms tumors. Clin Cancer Res. 2008;14:7635–44. https://doi.org/10.1158/1078-0432.CCR-08-1620.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  62. Hou LT, Holman RL. Bilateral nephroblastomasis in a premature infant. J Pathol Bacteriol. 1961;82:249–55. https://doi.org/10.1002/path.1700820202.

    Article  CAS  PubMed  Google Scholar 

  63. Perlman EJ, Faria P, Soares A, Hoffer F, Sredni S, Ritchey M, et al. Hyperplastic perilobar nephroblastomatosis: long-term survival of 52 patients. Pediatr Blood Cancer. 2006;46:203–21. https://doi.org/10.1002/pbc.20386.

    Article  PubMed  Google Scholar 

  64. Vicens J, Iotti A, Lombardi MG, Iotti R, De Davila MTG. Diffuse hyperplastic perilobar nephroblastomatosis. Pediatr Dev Pathol. 2009;12:237–8. https://doi.org/10.2350/07-09-0349.1.

    Article  PubMed  Google Scholar 

  65. Kilton L, Matthews MJ, Cohen MH. Adult Wilms tumor: a report of prolonged survival and review of literature. J Urol. 1980;124:1–5. https://doi.org/10.1016/s0022-5347(17)55264-7.

    Article  CAS  PubMed  Google Scholar 

  66. Szychot E, Apps J, Pritchard-Jones K. Wilms' tumor: biology, diagnosis and treatment. Transl Pediatr. 2014;3:12–24. https://doi.org/10.3978/j.issn.2224-4336.2014.01.09.

    Article  PubMed  PubMed Central  Google Scholar 

  67. Arda I, Tüzün M, Demirhan B, Sevmis S, Hicsönmez A. Lumbosacral extrarenal Wilms’ tumour: a case report and literature review. Eur J Pediatr. 2001;160:617–9. https://doi.org/10.1007/s004310100819.

    Article  CAS  PubMed  Google Scholar 

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Tanwar, P. (2022). Pathology. In: Sarin, Y.K. (eds) Wilms’ Tumor. Springer, Singapore. https://doi.org/10.1007/978-981-19-3428-5_7

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