Wilms’ Tumor in Adults—Conventional and Unconventional Presentations of a Rare Entity with a Review of Literature

Main Article Content

Sujata Tripathi
Amit Mishra
Vijay C Popat
Syed Altaf Husain


Adult wilm’s tumour, Inferior vena cava, nephroblastoma, NWTS, SIOP, Thrombus


Wilms’ tumor (WT) in adults is a rare neoplasm. Only a few reports are available in the literature. The tumor often masquerades as renal cell carcinoma (RCC). For accurate reporting, histopathological examination (HPE) plays a vital role in early diagnosis and prompt administration of multimodality treatment helps to improve the prognosis.
We comprehensively analyzed five cases of adult WT presenting in the third to fifth decade with flank pain, hematuria, fever, and palpable lump. After complete clinical, biochemical, radiological, and HPE evaluation, tumor was staged and treatment was planned accordingly.
Patients with low-stage WT were treated with open radical nephrectomy and chemotherapy. One of the patients diagnosed with inferior vena cava (IVC) thrombus apart from the above treatment also underwent IVC thrombectomy. Another young male presenting with distant metastasis (stage IV) and focal anaplasia on histology received preoperative chemotherapy and then planned for surgery. Unfortunately, the tumor being unresectable, second-line chemotherapy was given but he ultimately succumbed to death. All other patients are on regular follow-up and disease-free.
Adult nephroblastoma is a rare clinical entity with hostile behavior. The presence of IVC thrombus is not a contraindication to surgery. Although the management strategy as per pediatric protocol by the inclusion of multimodality approach improves survival, still the overall prognosis in adults is dismal. There is a need for a standardized treatment protocol to encourage a homogenous approach for this rare disease and thereby improve survival.

Abstract 38 | PDF Downloads 18 XML Downloads 9 HTML Downloads 8


1. Geethamani V, Kusuma V, Gowda KS, Saini ML. Adult Wilms’ tumour: A case report with review of literature. Diagn Pathol. 2006;1:46–8. https://doi.org/10.1186/1746-1596-1-46
2. Huff V. Wilms’ tumours: About tumour suppressor genes, an oncogene and a chameleon gene. Nat Rev Cancer. 2011;11(2):111–21. https://doi.org/10.1038/nrc3002
3. Patil TV, Patel KM, Shukla SN, Parikh BJ, Anand AS, Shah MS. Adult Wilms’ tumor. Case report. Indian J Med Pediatr Oncol. 2008;4:37–40. https://doi.org/10.4103/0971-5851.51407
4. Kattan J, Tournade MF, Culinde S, Terrier–Lacombe MJ, Droz JP. Adult Wilms’ tumor: Review of 22 cases. Eur J Cancer. 1994;30:1778–82. https://doi.org/10.1016/0959-8049(94)00315-V
5. Wszolek MF, Wotkowicz C, Libertino JA. Surgical management of large renal tumors. Nat Clin Pract Urol. 2008;5:35–46. https://doi.org/10.1038/ncpuro0963
6. Ciancio G, Soloway M. Resection of the abdominal IVC for complicated renal cell carcinoma with tumor thrombus. BJU Int. 2005;96:815–18. https://doi.org/10.1111/j.1464-410X.2005. 05719.x
7. Hentrich MU, Meister P, Brack NG, Lutz LL, Hartenstein RC. Adult Wilms’ tumor: Report of two cases and review of the literature. Cancer. 1995:75:545–51. https://doi.org/10.1002/1097-0142(19950115)75:2%3C545::AID-CNCR2820750218% 3E3.0.CO;2-C
8. Dreicer R, Donovan J, Benda JA, Lund J, Degowin RL. Paraneoplastic erythrocytosis in a young adult with an erythropoietin-producing Wilms’ tumor. Am J Med. 1992:93:229–30. https://doi.org/10.1016/0002-9343(92)90056-H
9. Wu J, Zhu Q, Zhu W, Chen W. CT and MRI imaging features and long-term follow-up of adult Wilms’ tumor. Acta Radiol. 2015;57(7):894–900. https://doi.org/10.1177/0284185115608658
10. Oto A, Herts BR, Remer EM, Novick AC. Inferior vena cava tumor thrombus in renal cell carcinoma: Staging by MR imaging and impact on surgical treatment. AJR Am J Roentgenol. 1998;171:1619–24. https://doi.org/10.2214/ajr.171.6.9843299
11. 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
12. Choi YJ, Jung WH, Shin DW, Park, Lyu CJ. Histopathological and immunohistochemical features of Wilms tumor. Korean J Pathol. 1993;27:339–48.
13. Huszno J, Starzyczny-Słota D, Jaworska M, Nowara E. Adult Wilms’ tumor—Diagnosis and current therapy. Cent European J Urol. 2013;66:39–44. https://doi.org/10.5173/ceju.2013.01.art12
14. Md Zin R, Murch A, Charles A. Pathology, genetics and cytogenetics of Wilms’ tumour. Pathology. 2011;43:302–12 https://doi.org/10.1097/PAT.0b013e3283463575
15. Kilton L, Mathews MJ, Cohen MH. Adult Wilms’ tumour: 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
16. Masuda H, Azuma H, Nakajima F, Watsuji T, Katsuoka  Y. Adult Wilms’ tumor with calcification untreated for five years – A case report. BMC Urol. 2004;4:5. https://doi. org/10.1186/1471-2490-4-5
17. Baba K, Yamaguchi O, Nomiya M, Hashimoto T, Yoshimura Y, Shiraiwa Y, et al. A case of adult Wilms’ tumor with vena caval involvement [in Japanese]. Hinyokika Kiyo. 1995;41:369–72. PMID: 7598037. Available at: http://hdl.handle.net/2433/115500
18. Martínez-Ibáñez V, Sánchez de Toledo J, De Diego M, Castellote A, Sábado C, Javier G, et al. Wilms’ tumor with intra-caval involvement. Med Pediatr Oncol. 1996;26:268–71. https://doi.org/10.1002/ (sici)1096-911x(199604)26:43.0.co;2-b
19. Mitchell C, Shannon R, Vujanic GM. The treatment of Wilms’ tumour: results of the United Kingdom Children’s cancer study group third Wilms’ tumour study. Med Pediatr Oncol. 2003;41:287. https://onlinelibrary.wiley.com/doi/epdf/10.1002/ mpo.10407
20. Bhatnager S. Management of Wilms’ tumor: NWTS vs SIOP. J Indian Assoc Pediatr Surg. 2009;14:6–14. https://doi.org/10.4103/0971-9261.54811
21. Sawicz–Birkowska K. Cze˛s´ciowa resekcja nerki (operacja oszc-ze˛dzaja˛ca narza˛du z wyboru) w leczeniu nerczaka. [Partial Nephrectomy (Nephron−Sparing Surgery) in the Treatment of Nephroblastoma]. Adv. Clin. Exp. Med. 2003;12(4):483–488.
22. de Kraker J, Graf N, van Tinteren H, Pein F, Sandstedt B, Godzinski J, et al. Reduction of postoperative chemotherapy in children with stage I intermediate risk and anaplastic Wilms, tumor (SIOP 93–01 trial) randomized controlled trial. Lancet. 2004;364:1229–35. https://doi.org/10.1016/ S0140-6736(04)17139-0
23. Vujanic GM, Sandstedt B, Harms D, Kelsey A, Leuschner I, de Kraker J. Revised International Society of Pediatric Oncology (SIOP) working classification of renal tumors of childhood. Med Pediatr Oncol. 2003;38:79–82. https://doi.org/10.1002/ mpo.1276
24. Kalapurakal JA, Nan B, Norkool P, Coppes M, Perlman E, Beckwith B, et al. Treatment outcomes in adults with favorable histologic type Wilms’ tumor–An update from the National Wilms Tumor Study Group. Int J Radiat Oncol Biol Phys. 2004;60(5):1379–84. https://doi.org/10.1016/j.ijrobp.2004.05.057
25. Reinhard H, Aliani S, Ruebe C, Stöckle M, Leuschner I, Graf N. Wilms’ tumor in adults: Results of the Society of Pediatric Oncology (SIOP) 93-01/Society for Pediatric Oncology and Hematology (GPOH) Study. J Clin Oncol. 2004;22:4500–6. https://doi.org/10.1200/JCO.2004.12.099
26. Terenziani M, Spreafico F, Collini P, Piva L, Perotti D, Podda M, et al. Adult Wilms tumor: A monoinstitutional experience and a review of the literature. Cancer. 2004;101:289–93. https://doi.org/10.1002/cncr.20387
27. Gooskens SL, Segers H, Pritchard-Jones K, Graf N, Jeffrey DS, van den Heuvel-Eibrink MM. The clinical relevance of age at presentation in nephroblastoma. In: van den Heuvel-Eibrink MM, editor. Wilms tumor. Brisbane, Australia: Codon Publications; 2016. p. 23–30. https://doi.org/10.15586/codon.wt.2016.ch2
28. Sghiri T, Ouafki I, Mouhtaram A, Bensouda Y, Mrabti H, Errihani H. Adult Wilms tumour: A rare presentation of two case reports with review of literature. J Clin Case Rep. 2013;3:277. https://doi.org/10.4172/2165-7920.1000277