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What is Neuroblastoma (Peripheral Neuroblastic Tumors)?
- The third most common pediatric malignancy after leukemia and brain tumors.
- The most common solid extra-cranial tumor in children
- More than 600 new cases are diagnosed in the US each year
- Neuroblastoma (NB) refers to a spectrum of peripheral neuroblastic tumors (PNT) that arises from the cell group differentiating to the autonomic nervus system in the neural crest.
- PNT primarily occurs in the adrenal medulla and sympathetic chains in the neck and thoracic and abdominal cavities and secretes catecholamine.
- 30% in the adrenal medulla
- 60% in paraspinal ganglia in the abdomen
- 10% in paraspinal ganglia in the chest, head/neck, and pelvis
Cheung NK, Dyer MA. Nat Rev Cancer. 2013 Jun;13(6):397-411.
Risk Stratification
- NB was once called an “enigmatic disease” because its clinical presentation is highly variable.
- Indolent disease (with spontaneous regression and maturation) to aggressive disease (with extensive local invasion, disseminated metastasis, and death)
- Clinical heterogeneity is associated with numerous clinical and biological prognostic factors.
Factors Used for Risk Stratification
- Age
- Tumor Stage
- Tumor histology based on “International Neuroblastoma Pathology Classification (INPC)”
- DNA ploidy (DNA index)
- MYCN gene amplification
- The prognostic factors are used for risk stratification
- Essential for determining the initial treatment regimen
1. Age
- Younger patients (under 12-18 months) have better outcomes.
2. Tumor Stage
- The stage represents the degree of local extension and metastasis of the tumor.
- Currently, two systems are used for NB risk stratification
- INRGSS: the International Neuroblastoma Risk Group Staging System
- Incorporates Image-defined risk factors (IDRF) based on the preoperative imaging results (e.g., CT, MRI, and MIBG scans).
- INSS: the International Neuroblastoma Staging System (INSS)
- Considers surgery results (complete vs. incomplete resection)
- INRGSS: the International Neuroblastoma Risk Group Staging System
3. Histologic Evaluation of Neuroblastoma
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4. DNA Ploidy
- Hyperploidy in NB is associated with a better prognosis, particularly in children younger than two years of age.
- DNA ploidy is not a good predictive factor for older patients.
5. MYCN Gene Amplification
- 20% of all neuroblastoma
- 45% of high-risk cases
- Highly associated with:
- Deletion of 1p especially del 1p36.3
- Elevated mRNA and protein expression
- Independently predicts treatment failure
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