Neuroblastoma Staging
International Neuroblastoma Staging System (INSS)
The staging of neuroblastoma is based on the International Neuroblastoma Staging System (INSS).[1]
| T |
Description
|
| Stage 1 |
- Localized tumor
- Complete gross excision
- Ipsilateral lymph nodes involvement negative under microscopic examination
|
| Stage 2A |
- Localized tumor
- Incomplete gross resection
- Ipsilateral lymph nodes involvement negative under microscopic examination
|
| Stage 2B |
- Localized tumor
- Complete or incomplete gross resection
- Ipsilateral lymph nodes involvement positive under microscopic examination
- Enlarged contralateral lymph nodes but with negative involvement under microscopic examination
|
| Stage 3 |
- Localized unilateral tumor with contralateral lymph nodes involvement positive under microscopic examination
or
- Unresectable unilateral tumor infiltrating across the midline with positive or negative regional lymph node involvement
or
- Unresectable midline tumor with bilateral infiltration or lymph node involvement
|
| Stage 4 |
- Metastasis of the tumor to distant lymph nodes
- Metastasis of the tumor to liver, skin, bone and/or other organs (except as defined by Stage 4S)
|
| Stage 4S |
- Limited to infants <1 year of age
- Localized primary tumor (as defined for stage 1, 2A, or 2B)
- Metastasis of the tumor limited to skin, liver and/or bone marrow
- Bone marrow involvement in stage 4S should be minimal (<10% of total nucleated cells identified as malignant on bone marrow biopsy)
|
International Neuroblastoma Risk Group Staging System (INRGSS)
| Stage: |
Description
|
| Stage L1 |
- Localized disease without image-defined risk factors
|
| Stage L2 |
- Localized disease with image-defined risk factors
|
| Stage M |
|
| Stage MS |
- Metastatic disease limited to skin, liver and/or bone marrow
- Bone marrow involvement in stage 4S should be minimal (<10% of total nucleated cells identified as malignant on bone marrow biopsy)
|
Risk Stratification
- Neuroblastoma patients are risk stratified into a low risk group, an intermediate risk group, and a high risk group based on the tumor INSS stage, the patient's age, and the tumor biology.