Causes of Fever in Children with Wilms Tumor
Fever in children with Wilms tumor is primarily caused by tumor-related systemic inflammatory responses, tumor necrosis releasing inflammatory mediators, and treatment-related complications including neutropenic fever during chemotherapy.
Tumor-Related Causes of Fever
- Systemic inflammatory response: Wilms tumor can trigger systemic inflammatory responses through release of cytokines and other inflammatory mediators
- Tumor necrosis: Spontaneous necrosis within the tumor mass can release cellular contents that trigger fever
- Tumor lysis syndrome: Rapid breakdown of tumor cells (spontaneous or treatment-induced) can cause metabolic derangements including fever 1
- Advanced disease: Children with metastatic disease may experience fever as a systemic symptom
Treatment-Related Causes of Fever
Chemotherapy-Induced Neutropenic Fever
- High-risk complication: Profound neutropenia with severe mucosal toxicity is common with intensive chemotherapy regimens used for Wilms tumor 1
- Timing: Typically occurs during nadir periods (lowest blood counts) after chemotherapy cycles
- Management approach: Requires prompt evaluation and empiric broad-spectrum antibiotics
Post-Surgical Fever
- Inflammatory response: Surgery for Wilms tumor (nephrectomy or nephron-sparing surgery) can trigger post-operative inflammatory responses
- Infection risk: Surgical site infections may develop, particularly with extensive procedures
Specific Fever Patterns
Fever with Lethargy and Rash
- A specific syndrome of fever accompanied by lethargy, irritability, and rash has been documented in patients undergoing multimodal treatment (surgery, radiation, and chemotherapy) for Wilms tumor 2
- This constellation of symptoms may represent a unique inflammatory response to treatment
Fever in DICER1 Syndrome
- Children with DICER1 syndrome have increased risk for Wilms tumor and may present with systemic symptoms including fever 1
- These patients require careful monitoring as they are at risk for multiple tumor types
Evaluation of Fever in Children with Wilms Tumor
Initial Assessment
- Complete blood count: To assess for neutropenia (ANC <500/mm³)
- Blood cultures: From all lumens of central venous catheters 1
- Consider peripheral blood cultures: Concurrent with central line cultures 1
- Urinalysis and culture: When clean-catch specimens are readily available 1
- Chest radiography: Only in symptomatic patients 1
Risk Stratification
- High-risk features: Profound neutropenia (ANC <500/mm³), clinical instability, mucositis, central venous catheter presence
- Low-risk features: Good clinical appearance, expected brief neutropenia, no comorbidities
Management Approach
For Neutropenic Fever
- High-risk patients: Use monotherapy with antipseudomonal β-lactam or carbapenem as empiric therapy 1
- Consider adding second agent: For clinically unstable patients or when resistant infection is suspected 1
- Duration: Continue antibiotics until patient has been afebrile for at least 24 hours and shows evidence of marrow recovery 1
For Non-Neutropenic Fever
- Evaluate for tumor progression: Consider imaging studies to assess disease status
- Rule out tumor lysis syndrome: Check electrolytes, renal function, and uric acid levels
- Supportive care: Antipyretics, hydration, and close monitoring
Prevention Strategies
- Infection prophylaxis: Consider antimicrobial prophylaxis during intensive chemotherapy phases
- Tumor lysis prevention: Hydration, allopurinol or rasburicase for patients at high risk
- Early intervention: Prompt recognition and management of fever to prevent complications
Special Considerations
- Bilateral Wilms tumor: These patients may have associated genetic syndromes and require more complex management 3
- Relapsed/refractory disease: More intensive therapy increases risk of fever and infectious complications 1
Understanding the various causes of fever in children with Wilms tumor is essential for appropriate management and prevention of complications that could impact morbidity and mortality.