International Neuroblastoma Staging System (INSS):
Stage I |
Localized tumor with complete gross excision with or without microscopic residual disease. Negative ipsilateral lymph nodes |
Stage 2A |
Localized tumor with incomplete gross resection. Negative ipsilateral lymph nodes |
Stage 2B |
Localized tumor with or without gross resection with positive ipsilateral lymph nodes. Negative contralateral lymph nodes |
Stage 3 |
Unresectable unilateral tumor infiltrating across the midline with or without regional lymph node involvement, localized unilateral tumor with contralateral lymph node involvement, midline tumor with bilateral extension by infiltration or by lymph node involvement |
Stage 4 |
Any primary tumor with dissemination to distant lymph nodes, bone, bone marrow, skin, liver, or other organs |
Stage 4S |
Localized primary tumor (as defined by stage 1, 2A, 2B) with dissemination limited to skin, liver, bone marrow (limited to infants < 1 yr of age) |
1. Multifocal primary tumors (e.g., bilateral adrenal primary tumors) should be staged according to the greatest extent of disease, as defined above, and followed by a subscript “M” (e.g. 3M).
2. The midline is defined as the vertebral column. Tumors originating on one side and crossing the midline must infiltrate to or beyond the opposite side of the vertebral column.
3. Marrow involvement in Stage 4S should be minimal, i.e., less than 10% of total nucleated cells identified as malignant on bone marrow biopsy or marrow aspirate. More extensive marrow involvement would be considered to be Stage 4. The MIBG scan (if performed) should be negative in the marrow.
4. Proven malignant effusion within the thoracic cavity if it is bilateral or the abdominal cavity upstages the patient to INSS 3.
Stage 4S
- Special assignment to children less than 1 year of age
- Metastases limited to skin, liver, and bone marrow biopsy
- May spontaneously regress
- Associated with good prognosis
Stage is an important predictor of outcome.
Stage |
5 - 10 year Survival |
1 |
Nearly all patients survive |
2 and 4S |
80% |
3 |
50 - 70% |
4 and over 1 year old |
Only 5 - 10% |
