Abstract
Purpose
The teratoid histologic variant of Wilms’ tumor is rare, with only 15 prior reported
cases. We review these and report an additional case in which a cytogenetic abnormality
was identified that has not previously been reported in a teratoid Wilms’ tumor.
Materials and methods
A medline search revealed 15 previously reported cases of the teratoid variant of
Wilms’ tumor. We summarized the characteristics of these cases with attention to radiologic
appearance, stage, laterality, histology, response to chemotherapy and outcomes.
Results
Characteristic radiologic features suggesting teratoid Wilms’ tumor were calcific
densities and stippling, or areas of attenuation indicating adipose tissue. The majority
of teratoid Wilms’ tumor patients had a high tumor stage at presentation (50% stage
III or greater). The incidence of bilateral tumors was 38%. Chemotherapy was administered
in nine cases and in only one (11%) was there a cytoreductive response. Four deaths
(25%) occurred amongst these patients.
Conclusions
Teratoid Wilms’ tumors appear to present with a high stage, increased incidence of
bilaterality and have a high mortality rate. Treatment strategies should focus on
total surgical extirpation, including metastatic sites when feasible, due to this
entity's limited response to chemotherapy.
Keywords
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Article Info
Publication History
Published online: January 22, 2007
Accepted:
November 21,
2006
Received:
August 29,
2006
Identification
Copyright
© 2006 Journal of Pediatric Urology Company. Published by Elsevier Inc. All rights reserved.
