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Wilms' tumor — treatment and prognosis of 373 children

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Summary

Between June 1980 and June 1988, 373 patients with Wilms' tumors were diagnosed and treated (34.5%, stage I; 34.5%, stage II; 14.8%, stage III; 10.7%, stage IV; and 5.4%, stage V). In all, 11% of the patients were diagnosed as a result of routine checkup examinations. The tumor volume was <400 ml in 47.3% of cases and >400 ml in 52.7%. Overall, 78.7% of the patients had a standard-type Wilms' tumor, 7% had a low-grade malignancy lesion and 14.3% had a high-grade-malignancy tumor. Aside from radical tumor nephrectomy, treatment encompassed chemotherapy in all cases and radiotherapy in half of the patients according to the study protocol. In all, 32% of the children received preoperative treatment and 68% underwent primary surgery. Overall, 81.8% (305/373) of the patients were disease-free according to the probability of disease-free survival after 6 years as calculated using the Kaplan-Meier method, with 90% of the protocol group (196/218) being free of disease. After primary surgery and subsequent chemotherapy, 97.3% of the children with standard-type Wilms' tumors of stage I were cured and 94% of those with stage II tumors survived disease-free, as did 88% of those in stage III, 46% of those in stage IV, and 75% of those in stage V (Kaplan-Meier method). Radiotherapy was performed in 113 of the 218 protocol patients. Children demonstrating a clear-cell histology subtype did not show the poor prognosis attributed to this histology in the literature. Of the children with low-grade variants, 19 of 24 are free of disease. About 70% of all children with Wilms' tumor in the Federal Republic of Germany were entered in this study (47 of probably 68 new cases recorded annually nationwide×8 years). Of the 47 patients evaluated per year, 38 survived disease-free, whereas 9 per year either suffered from a disease or perished. The overall results are very satisfying. Additional treatment modifications can be achieved only through further studies. Not only for this reason is it recommended that Wilms' tumors be treated at large institutions. Wilms' tumor is the most common solid childhood tumor; 6.5% of all malignancies occurring in children are nephroblastomas. About 70 new cases are diagnosed every year in the Federal Republic of Germany.

The therapy of Wilms' tumors depends on various factors such as the clinical stage and histologic subtype of the tumor and the age of the patient. New information for optimization of the therapy can only be gathered by means of a nationwide study. We report on the clinical data and treatment results of 373 children. The aim of this study was to elucidate the relationship between the prognosis and the clinical stage and histologic subtype of the tumor as well as the age of the patients. In addition, the importance of pre- and/or postoperative radiotherapy was studied. Furthermore, the dose and duration of treatment with chemotherapeutic agents was evaluated.

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Authors and Affiliations

  1. Department of Urology, University of Mainz, Medical School, W-6500, Mainz

    R. Wammack, M. Fisch & R. Hohenfellner

  2. Department of Paediatrics, University of Mainz, Medical School, W-6500, Mainz

    P. Gutjahr

  3. Prof. Hess-Kinderklinik, Bremen

    H. J. Spaar

  4. Department of Paediatrics, University of Würzburg, Federal Republic of Germany

    J. Kühl

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  1. R. Wammack
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  2. M. Fisch
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  4. J. Kühl
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  5. P. Gutjahr
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  6. R. Hohenfellner
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Wammack, R., Fisch, M., Spaar, H.J. et al. Wilms' tumor — treatment and prognosis of 373 children. World J Urol 10, 100–106 (1992). https://doi.org/10.1007/BF00183143

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