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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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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DOI: https://doi.org/10.1007/s00120-007-1363-0