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Aktuelle Urol 1991; 22(2): 75-78
DOI: 10.1055/s-2008-1060482
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© Georg Thieme Verlag, Stuttgart · New York

Wilms-Tumor-Varianten niedrigen Malignitätsgrades 24 Fälle aus der kooperativen Wilms-Tumor-Studie 1980-88

Low-Grade Malignancy Variants of Wilms' Tumor A Report of 24 Cases From a German Cooperative Study Group (1980-88)R. Kühn, R. Hohenfellner, P. Gutjahr
  • Universitätskliniken Mainz
Für die freundliche Überlassung von Patientendaten danken wir Prof. C. Eschenbach (Marburg), Dr. Fengler (Berlin-Charlottenburg), Dr. Gloöckel (Erlangen), Dr. N. Graf (Homburg/S.), Prof. U. Goöbel (Düsseldorf), Dr. Astrid Gnekow (Augsburg), Dr. J. Kühl (Würzburg), Prof. R. Ludwig (Heidelberg), Dr. G. Nessler (Karlsruhe), Prof. D. Niethammer (Tübingen), Dr. Schöck (Bielefeld-Bethel), Dr. H. J. Spaar (Bremen)
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Publication History

Publication Date:
28 April 2008 (online)

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Zusammenfassung

In einer bundesweiten Wilms-Tumor-Studie (5) fanden sich bei 24 von 342 Patienten (= 7 %) sogenannte niedrigmaligne Wilms-Tumor-Varianten, davon 15 kongenitale mesoblastische Nephrome (KMN), 4 fetale rhabdomyomatoöse Nephrome (FRN) und 5 zystische, partiell differenzierte Nephrome (ZPDN). Diese Kinder waren durchschnittlich juünger als die mit histologischem Standardtyp. Die initiale Therapie dieser Tumoren ist operativ, eine kurzdauernde postoperative Chemotherapie kann beim FRN sinnvoll sein. Die Prognose ist gut.

Abstract

Among 342 Patients of a German Cooperative Wilms' Tumor Study from 1980 to 1988 there were 24 who had variants of low grade malignancy. 15 of these were histologically classified as congenital mesoblastic nephroma (KMN), 4 as fetal rhabdomyomatous nephroma (FRN) and 5 as cystic, partially differentiated nephroma. The patients were younger than those with Wilms' tumors of standard malignancy. KMN occurred at a lower age than FRN and ZPDN. The variants of low malignancy presented mostly with an asymptomatic tumor mass. One FRN occurred synchronously bilateral, one ZPDN (first case described so far) was metachronously bilateral. Surgical therapy alone is sufficient for KMN and ZPDN, for FRN additional chemotherapy may be recommended. The patient with the bilateral FRN died from tumor progression, 3 patients died after surgical treatment for the primary tumor.

Key-Words

Wilms' Tumor - Congenital mesoblastic nephroma - Fetal rhabdomyomatous nephroma - Cystic, Partially differentiated nephroma

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