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Wilms-Tumor bei Erwachsenen

Wilms’ tumor in adults

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Zusammenfassung

Hintergrund

Der Wilms-Tumor (Nephroblastom) ist der häufigste Nierentumor bei Kindern. Bei Erwachsenen ist das Nephroblastom selten. Bisher hatte die Erkrankung eine schlechte Prognose.

Patienten und Methoden

Im Rahmen der pädiatrischen Nephroblastomstudie konnten wir von 1994–2005 bei insgesamt 1300 Neumeldungen 41 Patienten >16 Jahre mit einem Wilms-Tumor registrieren. Das mittlere Alter bei Diagnose betrug 25,4 (16,0–62,0) Jahre. Die Therapie wurde entsprechend dem pädiatrischen Protokoll gegeben.

Ergebnisse

Die Erwachsenen hatten höhere lokale Stadien, häufiger Metastasen und zeigten eine stärkere Toxizität auf die Therapie. Vor allem die Gabe von Vincristin führte zu schwerer Neurotoxizität. Die Verteilung der histologischen Subtypen entsprach der des Kindesalters. Entgegen den bisher beschriebenen schlechten Behandlungsergebnissen betrug das Gesamtüberleben in dieser Kohorte 71%. Patienten mit lokalem Stadium I und II haben ein mit Kindern vergleichbares ereignisfreies Überleben von 84%.

Schlussfolgerung

Erwachsene mit Nephroblastom haben eine sehr gute Heilungschance, wenn sie nach einem pädiatrischen Protokoll behandelt werden.

Abstract

Background

Wilms’ tumor (nephroblastoma) is the most frequent renal tumor in childhood. In contrast nephroblastoma in adults is rare, and the disease used to have a poor prognosis.

Patients and methods

Of 1,300 registered patients, a total of 41 patients older than 16 years were enrolled in the pediatric nephroblastoma trial from 1994 to 2005. Median age at diagnosis was 25.4 years (range: 16–62 years). Treatment was given according to the pediatric protocol.

Results

The adults had higher local stages, more frequent metastasis, and developed more toxicity due to therapy. Vincristine caused severe neurotoxicity in many cases. The distribution of histological subtypes was similar to the children’s. The outcome is better than previously described with an overall survival of 71%. Patients with local stage I and II have an event-free survival of 84%. This is comparable to children’s survival rates.

Conclusion

Adults with nephroblastoma have a very good prognosis if treated according to a pediatric protocol.

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Literatur

  1. Akmansu M, Yapici T, Tülay E (1998) Adult Wilms‚Tumor. A report of two cases and their treatment and prognosis. Int J Urol Nephrol 30: 529–533

    Article  CAS  Google Scholar 

  2. Arrigo S, Beckwith JB, Sharples K et al. (1990) Better survival after combined modality care for adults with Wilm‚s tumor. Cancer 66: 827–830

    Article  PubMed  CAS  Google Scholar 

  3. Bailey LE, Durkee CD, Werner AL, Finley RK (1987) Wilms‚Tumor in adults. Am Surg 5: 149–155

    Google Scholar 

  4. Butler D, Buchino JD, Jose B et al. (1997) Wilms‚Tumor in an adult: a case report and review of literature. KMA J 95: 191–196

    CAS  Google Scholar 

  5. Bozeman G, Bissada NK, Abboud MR et al. (1995) Adult Wilms‚Tumor: prognostic and management considerations. Urology 45: 1055–1058

    Article  PubMed  CAS  Google Scholar 

  6. Byrd R, Evans E, D‘Angio D (1982) Adult Wilms‚Tumor: effect of combined therapy on survival. J Urol 127: 648–651

    PubMed  CAS  Google Scholar 

  7. Faria P, Beckwith JB, Mishra K et al.(1996) Focal versus diffuse anaplasia- new definitions with prognostic significance: A report from the National Wilms‚Tumor Study Group. Am J Surg Pathol 20: 909–920

    Article  PubMed  CAS  Google Scholar 

  8. Graf N, Tournade MF, Kraaker J de (2000) The role of preoperative chemotherapy in the management of Wilms‚Tumor.The SIOP studies. Urol Clin North Am 27: 443–450

    Article  PubMed  CAS  Google Scholar 

  9. Green DM, Breslow NE, Beckwith JB et al. (1998) Effect of duration of treatment on treatment outcome and cost of treatment for Wilms‚Tumor: a report from the National Wilms‚Tumor Study Group. J Clin Oncol 16: 3744–3751

    PubMed  CAS  Google Scholar 

  10. Gutjahr P (1980) Results of treatment of Wilms‚Tumors according to a plan (I/76) of the Society of Pediatric Oncology-a retrospective analysis. Klin Padiatr 192: 109–116

    Article  PubMed  CAS  Google Scholar 

  11. Gutjahr P, Neisius D, Bode U et al. (1992) Therapie und Prognose bei 11 Erwachsenen mit Wilms-Tumoren (Nephroblastome). Akt Urol 23: 232–235

    Google Scholar 

  12. Huser J, Grignon DJ, Ro JY et al. (1990) Adult Wilms‚Tumor: a clinicopathologic study of 11 cases. Modern Pathol 3: 321–326

    CAS  Google Scholar 

  13. Kalapurkal JA, Nan B, Norkol P et al. (2004) Treatment outcome in adults with favorable histologic type Wilms‚Tumor-An update from the National Wilms Tumor Group. Int J Radiol Oncol Biol Phys 60: 1379–1384

    Article  Google Scholar 

  14. Kattan J, Tournade MF, Culine S et al. (1994) Adult Wilms‚Tumour: review of 22 cases. Eur J Cancer A 30: 1778–1782

    Article  Google Scholar 

  15. Kinderkrebsregister (1999) Jahresbericht 1999. Kinderkrebsregister, Universität Mainz

  16. Knispel H, Dieckmann KP, Henze G et al. (1990) Wilms-Tumor beim Erwachsenen. Urologe A 29: 226–229

    PubMed  CAS  Google Scholar 

  17. Lurie M, Sova I, Mecz Y et al. (1988) Adult nephroblastoma. Cancer 61: 2342–2347

    Article  PubMed  CAS  Google Scholar 

  18. Meyer JS, Harty MP, Khademian Z (2002) Imaging of neuroblastoma and Wilms‚Tumor. Magn Reson Imag Clin North Am 10: 275–302

    Article  Google Scholar 

  19. Mydlo JH, Horowitz M, delRosario C et al. (1996) An unusual presentation of adult Wilms‚Tumor. Urol Int 57: 104–107

    PubMed  CAS  Google Scholar 

  20. National Cancer Institute (1999) Common Toxicity Criteria (CTC), Version 2.0. National Cancer Institute, Rockville, USA

  21. Reinhard H, Semler O, Burger D et al. (2004) Results of the SIOP 93–01/GPOH trial and study for the treatment of patients with unilateral nonmetastatic Wilms‚Tumor. Klin Padiatr 216: 132–140

    Article  PubMed  CAS  Google Scholar 

  22. Reinhard H, Aliani S, Ruebe C et al. (2004) Wilms‚Tumor in adults: Results of the society of pediatric oncology (SIOP) 93–01/GPOH for pediatric oncology and hematology (GPOH) study. JCO 22: 4500–4506

    Article  Google Scholar 

  23. Schenk JP, Schrader C, Zieger B et al. (2006) Reference radiology in nephroblastoma: accuracy and relevance for preoperative chemotherapy. Rofo 178: 38–45

    PubMed  Google Scholar 

  24. Sparano JA, Beckwith B, Mitsudo S, Wiernik PH (1991) Complete remission in refractory anaplstic adult Wilms‚Tumor treated with cisplatin and etoposide. Cancer 67: 956–959

    Article  PubMed  CAS  Google Scholar 

  25. Ternziani M, Spreafico F, Collini P et al.(2004) Adult Wilms‚Tumor: A monoinstitutional experience and review of literature. Cancer 101: 289–293

    Article  Google Scholar 

  26. Tournade MF, Com-Nougue C, Voute PA et al.(2001) Optimal duration of preoperative therapy in unilateral and nonmetastatic Wilms‚Tumor in children older than 6 months: Results of the SIOP 9 study and trial. J Clin Oncol 19: 488–500

    PubMed  CAS  Google Scholar 

  27. Vujanić GM, Sandstedt B, Harms D et al. (2002) Revised International Society of Paediatric Oncology (SIOP) working classification of renal tumors of childhood. Med Pediatr Oncol 38: 79–82

    Article  PubMed  Google Scholar 

  28. Weichert-Jacobsen K, Papadopoulos I, Skrezek C et al. (1995) Adult Wilms‚Tumor: Case report, management, prognosis. Urol Int 54: 99–103

    Article  PubMed  CAS  Google Scholar 

  29. Williams G, Colbeck RA, Gowing NFC (1992) Adult Wilms‚Tumour: Review of 14 patients. Br J Urol 70: 230–235

    PubMed  CAS  Google Scholar 

  30. Winter P, Vogel J, Schoeneich G et al. (1990) Das Nephroblastom im Erwachsenenalter. Urologe A 29: 219–225

    PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Klinik für Pädiatrische Hämatolgie und Onkologie, Universitätsklinikum, 66421, Homburg/Saar, Deutschland

    H. Reinhard, R. Furtwängler & N. Graf

  2. Klinik für Urologie und Kinderurologie, Universitätsklinikum, Homburg/Saar, Deutschland

    S. Siemer & B. Wullich

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  1. H. Reinhard
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  2. R. Furtwängler
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  3. S. Siemer
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  4. B. Wullich
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  5. N. Graf
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Correspondence to H. Reinhard.

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Reinhard, H., Furtwängler, R., Siemer, S. et al. Wilms-Tumor bei Erwachsenen. Urologe 46, 748–753 (2007). https://doi.org/10.1007/s00120-007-1363-0

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