Journal of Clinical Oncology, Vol 7, 310-315, Copyright © 1989 by American Society of Clinical Oncology

ARTICLES

Bilateral Wilms' tumor: long-term survival and some epidemiological features

MJ Coppes, J de Kraker, PJ van Dijken, HJ Perry, JF Delemarre, MF Tournade, J Lemerle and PA Voute
Netherlands Cancer Foundation, Utrecht.

Sixty-seven children with a bilateral Wilms' tumor (BWT) who were registered to the International Society of Pediatric Oncology (SIOP) nephroblastoma trial and studies 1, 2, and 5, conducted between 1971 and 1980, were analyzed. The overall 10-year survival was 64%. While most deaths due to tumor occurred within 3 years after diagnosis of bilateral disease, five patients died after 3 years (20%), three with synchronous and two with metachronous BWT. The survival rates for the 42 children with synchronous BWT (follow-up time, 6 1/2 to 14 years) and the 25 children with metachronous BWT (follow-up time, 5 to 13 years) were 69% and 56%, respectively. Due to an improvement in the synchronous group, overall survival improved over the years: 47%, 72%, and 70%, in SIOP 1, 2, and 5, respectively. Age at diagnosis and most advanced tumor stage affected prognosis. Children presenting a tumor manifestation before the age of 2 years had better prognosis than older children. This difference is significant in synchronous BWT. Prognosis for children with local stage 1 or 2 was better than for those with local stage 3. Median age at initial presentation in BWT was lower than in unilateral nephroblastoma and lower in metachronous BWT than in synchronous BWT. Young children presenting with unilateral nephroblastoma should have a careful follow-up of the contralateral kidney for at least the next 3 1/2 years, as most contralateral tumors will develop during this period.
CiteULike    Complore    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this?

This article has been cited by other articles:

				M. L. Metzger and J. S. Dome Current Therapy for Wilms' Tumor Oncologist, November 1, 2005; 10(10): 815 - 826. [Abstract] [Full Text] [PDF]

				A. Koziell and R. Grundy Frasier and Denys-Drash syndromes: different disorders or part of a spectrum? Arch. Dis. Child., October 1, 1999; 81(4): 365 - 369. [Full Text]

				M. J. Coppes, D. A. Haber, and P. E. Grundy Genetic Events in the Development of Wilms' Tumor N. Engl. J. Med., September 1, 1994; 331(9): 586 - 590. [Full Text]