Original Paper

Urological Research

, Volume 25, Issue 4, pp 223-230

First online:

Progress of fundamental research in Wilms' tumor

  • J. G. WenAffiliated withDivision of Urological Oncology, Medical Faculty, Erasmus University Rotterdam
  • , G. J. van SteenbruggeAffiliated withDivision of Urological Oncology, Medical Faculty, Erasmus University Rotterdam
  • , R. M. EgelerAffiliated withDepartment of Pediatric Oncology, Sophia Children's Hospital
  • , R. M. NijmanAffiliated withDepartment of Pediatric Urology, Institute of Urology, Erasmus University and Sophia Children's Hospital Rotterdam

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The progress of fundamental research on the histopathological and molecular genetic properties, model systems, growth factor involvement, and tumor markers of clinical nephroblastoma (Wilms' tumor) are reviewed. Histologically, Wilms' tumor (WT) has been found to reveal a disorganized renal developmental process in which blastema and epithelia are randomly interspersed in varying amounts of stroma. Anaplasia is the only criterion for assigning a WT as having an “unfavorable histology.” Cytogenetic analysis identified WT genes at chromosome 11p13 (WTI), 11p15 region (WT2), and 16q (WT3). Permanent in vitro WT cell lines and in vivo WT models, such as human xenografts, have been established which provide indefinite sources of tumor material for fundamental, as well as therapy-directed, research. Abnormalities of growth factor (GF) expression in WT indicate that GF may play an important role in WT pathogenesis. A series of monoclonal antibodies was tested in WT by immunohistochemical techniques to identify specific diagnostic and prognostic markers. p53 expression in anaplastic WT is significantly higher than in differentiated WTs, indicating p53 may be a prognostic marker. Although significant progress has been made in the fundamental research, our basic knowledge of this malignancy is still limited. The availability of suitable experimental models, particularly the human xenograft system, offers the opportunity for further study of the cell biological and molecular aspects of WT and its clinical progression.

Key words

Wilms' tumor Histopathology Experimental model Tumor markers Growth factors