H Reinhard1, R Furtwängler, S Siemer, B Wullich, N Graf. 1. Klinik für Pädiatrische Hämatolgie und Onkologie, Universitätsklinikum, 66421, Homburg/Saar. harald.reinhard@uniklinikum-saarland.de
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.
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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