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Survival in nephroblastoma treated according to the trial and study SIOP-9/GPOH with respect to relapse and morbidity
1Department of Pediatric Oncology, University of Heidelberg, Im Neuenheimer Feld 150, D-69120 Heidelberg, Germany. angela_weirich@med.uni-heidelberg.de
Background:
Recent Wilms' tumor (WT) trials and studies have tried to determine the minimal therapy needed for cure. The goal was survival without morbidity.
Patients And Methods:
From January 1989 to March 1994 the German Society of Pediatric Oncology and Hematology registered 440 patients (median age 2.9 years; 231 male, 209 female) with WTs (preoperative chemotherapy 362) for therapy according to the International Society of Pediatric Oncology Trial and Study 9. Therapy for relapse depended on site of relapse and therapy already received. Follow-up included inquiries for morbidity. Prognostic factors for relapse and death were evaluated.
Results:
Five-year survival of WTs was 89.5%; 98.2% (385 of 392) of survivors had a follow-up of 5 years (range 0.8-12.6; median 8). In non-anaplastic WTs, young age (<2 years) was of significance (P = 0.026) for a better survival. Non-anaplastic WTs (407 patients) had a 5-year survival of 92.3%, versus 48.5% in anaplastic WTs (33 patients), and a 5-year relapse-free survival of 87.6% versus 42.4%. Survival after relapse was significantly worse for anaplastic than for non-anaplastic WTs (residual 3-year survival 11.8% versus 54.3%; P <0.0001). In preoperatively treated WTs, anaplasia was a strong prognostic factor for death [relative risk (RR) 4.7], followed by poor response to preoperative therapy (RR 3.6), stage IV (RR 3.2) and abdominal stage III (RR 2.2). Low abdominal stages (
Conclusions:
Initial therapy should be more individualized, taking the above risk groups (age in non-anaplastic WTs, poor response, anaplasia, etc.) into account, as morbidity even after relapse therapy with ifosfamide, carboplatin and etoposide was not high. Milder therapy in low stages of differentiated and of well responding WTs should be tested.
Insights
Wilms tumor treatment can be individualized based on risk factors like age and anaplasia. Minimizing therapy for low-risk Wilms tumor patients may reduce long-term side effects without compromising survival.
Area of Science:
- Pediatric Oncology
- Nephrology
- Clinical Trials
Background:
- Wilms tumor (WT) treatment aims for cure with minimal morbidity.
- Recent trials focus on identifying the least intensive effective therapy for WT.
Purpose of the Study:
- To evaluate prognostic factors for survival and relapse in Wilms tumor patients.
- To assess the impact of therapy on survival and long-term morbidity.
Main Methods:
- A cohort of 440 Wilms tumor patients (median age 2.9 years) were treated per SIOP Trial and Study 9 guidelines.
- Therapy for relapse was individualized; follow-up included morbidity assessment.
- Prognostic factors for relapse and death were analyzed.
Main Results:
- Five-year survival for Wilms tumor was 89.5%.
- Anaplastic histology (48.5% survival) significantly worsened outcomes compared to non-anaplastic WTs (92.3% survival).
- Younger age (<2 years) improved survival in non-anaplastic WTs. Morbidity was low (6.4%) at follow-up.
Conclusions:
- Individualized Wilms tumor therapy is recommended, considering risk factors like anaplasia and patient age.
- Milder treatment strategies for low-stage, differentiated, or well-responding WTs warrant investigation to reduce long-term morbidity.
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