Initial Treatment for Wilms Tumor
The initial treatment for a patient diagnosed with Wilms tumor consists of neoadjuvant chemotherapy followed by surgical resection, typically through radical nephrectomy, as part of a multi-phase, combination chemotherapy regimen. 1
Diagnostic Confirmation and Staging
Before initiating treatment, proper diagnosis and staging are essential:
- Complete imaging workup including:
- Abdominal ultrasound
- CT scans of abdomen, pelvis, chest
- Consider MRI for better delineation of tumor extension
- Genetic testing for predisposition syndromes (WT1, REST, TRIM28, DIS3L2) 2
- Laboratory studies including complete blood count, renal function tests
Treatment Algorithm
Step 1: Neoadjuvant Chemotherapy
- Standard approach in most cases to reduce tumor size before surgery
- Typically includes Actinomycin D at 45 mcg/kg intravenously once every 3-6 weeks 1
- Often combined with vincristine as part of multi-agent regimen
- Duration: Usually 4-6 weeks before surgical intervention
Step 2: Surgical Resection
- Radical nephrectomy remains the standard surgical approach
- Must include:
- Complete tumor removal with intact capsule (avoid tumor rupture)
- Regional lymph node sampling (essential for accurate staging)
- Examination of contralateral kidney
- Special considerations:
Step 3: Adjuvant Therapy
- Based on surgical findings and pathology:
- Favorable histology: Continue chemotherapy
- Unfavorable histology or advanced stage: Intensified chemotherapy plus radiation therapy
- Typical duration: Up to 26 weeks total 1
Special Considerations
Bilateral Wilms Tumor
- Requires more conservative surgical approach
- Bilateral partial nephrectomies or unilateral nephrectomy with contralateral partial nephrectomy 5
- More intensive preoperative chemotherapy to reduce tumor size
Genetic Predisposition Syndromes
- Patients with WT1, REST, TRIM28, or DIS3L2 mutations require careful surveillance and may need modified treatment approaches 2
- Consider genetic counseling for family members
Complications Management
- Monitor for tumor lysis syndrome during initial treatment
- Assess for myelosuppression before each chemotherapy cycle
- Manage fever according to oncology protocols, with high suspicion for neutropenic fever 6
Treatment Efficacy and Prognosis
Five-year survival exceeds 90% for children with favorable histology Wilms tumor who receive appropriate treatment 7. The excellent outcomes underscore the importance of prompt initiation of the standard treatment protocol with a multidisciplinary team experienced in managing pediatric renal tumors.
Pitfalls to Avoid
- Delaying surgical consultation after diagnosis
- Inadequate lymph node sampling during surgery
- Tumor rupture during resection (associated with increased recurrence risk)
- Failure to consider bilateral disease or predisposition syndromes
- Inappropriate dose reduction of chemotherapy without valid clinical reason
The treatment approach should be coordinated by a multidisciplinary team with experience in managing renal tumors in children, with consultation from pediatric oncology, surgery, radiation oncology, and pathology specialists.