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Bilateral Wilms' tumors with progressive or nonresponsive disease
Robert C Shamberger1, Gerald M Haase, Pedram Argani
1Department of Surgery, Children's Hospital Boston and Harvard Medical School, Boston, MA 02115, USA. robert.shamberger@childrens.harvard.edu
Journal of Pediatric Surgery
|March 29, 2006
Summary
Children with bilateral Wilms' tumors (BWT) and progressive or nonresponsive disease (PNRD) often received prolonged chemotherapy. Early biopsies are crucial to identify anaplastic tumors needing nephrectomy or differentiated tumors requiring early resection.
Area of Science:
- Pediatric Oncology
- Nephrology
- Surgical Oncology
Background:
- Bilateral Wilms' tumors (BWT) represent a significant challenge in pediatric oncology.
- The National Wilms Tumor Study-4 (NWTS-4) provides valuable data on treatment outcomes.
- Understanding outcomes for progressive or nonresponsive disease (PNRD) in BWT is critical for refining treatment protocols.
Purpose of the Study:
- To establish guidelines for future cooperative group trials involving BWT.
- To analyze the treatment and outcomes of children with BWT experiencing PNRD.
- To identify optimal management strategies for refractory BWT.
Main Methods:
- Retrospective review of treatment and outcome data from NWTS-4.
- Analysis of 38 children with BWT and PNRD.
- Evaluation of preoperative chemotherapy duration, subsequent regimens, and irradiation in BWT patients.
Main Results:
- Children with BWT and PNRD underwent prolonged preoperative chemotherapy (median 7 months), with many receiving second or third regimens.
- Anaplastic histology was identified in 4 patients, some developing it after initial therapy.
- Other findings included rhabdomyomatous or differentiated stromal elements and complete necrosis.
Conclusions:
- Prolonged chemotherapy is common for BWT patients with PNRD.
- Early and sequential biopsies are essential to guide treatment decisions.
- Biopsies can differentiate anaplastic tumors (best managed with early nephrectomy and intensive chemotherapy) from differentiated tumors (best managed with early resection).
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