Teratoid Wilms’ tumor, an important variant of nephroblastoma

Myers, Jeremy B.; Dall'Era, Joseph; Odom, Lorrie F.; McGavran, Loris; Lovell, Mark A.; Furness, Peter

doi:10.1016/j.jpurol.2006.11.004

« Previous Next »Journal of Pediatric Urology
Volume 3, Issue 4 , Pages 282-286, August 2007

Teratoid Wilms’ tumor, an important variant of nephroblastoma

Jeremy B. Myers
- Department of Urology, The Children's Hospital, 1056 E. 19th Ave, Box 463, Denver, CO 80218, USA
- Corresponding author. Tel.: +1 303 861 6846; fax: +1 303 864 5572.
Joseph Dall'Era
- University of Colorado Health Sciences Center, Division of Urology, 9th Ave, Mail Stop C302, Denver, CO 80206, USA
Lorrie F. Odom
- Department of Urology, The Children's Hospital, 1056 E. 19th Ave, Box 463, Denver, CO 80218, USA
Loris McGavran
- Department of Urology, The Children's Hospital, 1056 E. 19th Ave, Box 463, Denver, CO 80218, USA
Mark A. Lovell
- Department of Pathology, The Children's Hospital, 1056 E. 19th Ave, Box 463, Denver, CO 80218, USA
Peter Furness III
- Department of Urology, The Children's Hospital, 1056 E. 19th Ave, Box 463, Denver, CO 80218, USA

Received 29 August 2006; accepted 21 November 2006. published online 22 January 2007.

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: Wilms' tumor, Nephroblastoma, Teratoid, Metastasis, Genetics, 1q