Neuroblastoma Risk Stratification Tool

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Free Neuroblastoma Risk Calculator – Pediatric Oncology Risk Stratification Tool

What Is Neuroblastoma?

Neuroblastoma is a pediatric solid tumor arising from immature nerve cells of the sympathetic nervous system. It accounts for 6-10% of childhood cancers and 15% of pediatric cancer deaths. This embryonal malignancy primarily affects children under 5 years, with median diagnosis at 17 months. Its clinical behavior varies dramatically—from spontaneous regression to lethal metastasis—making risk stratification critical for treatment decisions.

Key Biological Drivers

Neuroblastoma’s heterogeneity stems from distinct molecular features:

• MYCN oncogene amplification (20% of cases; poor prognosis)

• Chromosome 11q deletion (aggressive disease marker)

• DNA ploidy status (hyperdiploidy favors survival in infants)

• ALK mutations (hereditary predisposition)

Why Risk Stratification Matters

The Children’s Oncology Group (COG) classifies neuroblastoma into three risk groups guiding therapy intensity:

Risk Group5-Year SurvivalTreatment ApproachLow Risk>95%Observation/surgeryIntermediate Risk90-95%Moderate chemoHigh Risk40-50%Multimodal intensive therapy

Table: Survival rates by COG risk stratification

Critical Stratification Factors

Our calculator evaluates these prognostic determinants:

1. Age at diagnosis (<18 months favors survival)

2. INSS Stage (International Neuroblastoma Staging System)

3. Histopathology (Shimada classification: favorable/unfavorable)

4. MYCN status (amplified = high risk)

5. DNA ploidy (hyperdiploid vs. diploid)

6. 11q chromosomal status

7. Metastatic burden (bone, marrow, liver involvement)

How the Neuroblastoma Risk Calculator Works

This evidence-based tool applies COG risk algorithms to your clinical inputs:

Input Parameters Explained

1. Tumor Characteristics

   • Primary site (adrenal vs. non-adrenal)

   • INSS Stage (1, 2A, 2B, 3, 4, 4S)

   • Metastatic sites (bone, marrow, liver)

2. Biological Markers

   • MYCN status: Amplification triples relapse risk

   • Histopathology: Unfavorable if >1.5% mitosis-karyorrhexis index

   • DNA Ploidy: Hyperdiploidy (DNA index >1) improves infant outcomes

3. Clinical Factors

   • Age in months (critical for Stage 4S infants)

   • Serum LDH/ferritin (elevated = poor prognosis)

Interpreting Your Results

The calculator outputs three components:

1. Risk Category

Color-coded visualization of low, intermediate, or high risk:

• Green (Low): 95% survival; surgery ± observation

• Yellow (Intermediate): 90% survival; 4-8 chemo cycles

• Red (High): 40-50% survival; stem cell transplant/immunotherapy

2. Clinical Recommendations

Personalized management strategies:

- Low Risk: • Monthly abdominal ultrasound × 3 months • Urinary catecholamines quarterly - High Risk: • GD2 immunotherapy (dinutuximab) • 13-cis-retinoic acid maintenance • Tumor sequencing for ALK inhibitors

3. Risk Meter Visualization

A sliding scale positions the patient’s risk relative to COG benchmarks:
|-----Low-----|----Intermediate----|--High--|
▲ Your result

Beyond Stratification: Emerging Biomarkers

While the calculator uses validated COG factors, novel markers are refining precision:

Next-Generation Prognostic Tools

• Transcriptome profiling (e.g., PAM32 gene signature)

• Circulating tumor DNA (detects MYCN via liquid biopsy)

• Immunohistochemical markers (PHOX2B, TH expression)

International Risk Systems Comparison

SystemKey FeaturesRegions UsedCOGTreatment-driven stratificationNorth AmericaINRGImage-defined risk factorsEuropeAJCCTNM-based stagingGlobal

Limitations and Clinical Validation

This tool adheres to COG ANBL0532 protocol criteria. Important considerations:

• Not diagnostic: Requires pathological confirmation

• Dynamic risk: Mid-treatment response (e.g., MIBG scan) may upgrade risk

• Genetic exceptions: PHOX2B or ALK-mutant cases need specialist review

Frequently Asked Questions

Why is age <18 months significant?

Infants exhibit unique biology: higher hyperdiploidy, spontaneous regression in Stage 4S, and responsiveness to chemo.

How does MYCN change management?

MYCN amplification mandates high-risk protocols regardless of stage.

Can Stage 4S be low risk?

Yes—if age <12 months, non-amplified MYCN, and hyperdiploid DNA.

Conclusion

Neuroblastoma risk stratification directly determines therapeutic intensity—from observation to stem cell transplantation. Our free neuroblastoma risk calculator synthesizes 10+ clinical variables into actionable risk categories using validated COG algorithms. By inputting tumor biology, stage, and age, clinicians receive instant risk categorization with evidence-based management recommendations. For high-risk results, prompt referral to pediatric oncology centers is advised.

Disclaimer: This tool supports clinical decisions but doesn’t replace specialist consultation.