Table 1.
Treatment and prognosis of neuroblastoma patients by risk group and staging. Prognosis is 5-year event-free survival [3,9,11,15,17]. MYCN: V-Myc avian myelocytomatosis viral oncogene neuroblastoma derived homolog.
| Risk Group | International Neuroblastoma Staging System (INSS) | Tumor Localization | Characteristics | Treatment | Prognosis (5 Year Event Free Survival) |
|---|---|---|---|---|---|
| Low | 1–2 |  |
Stage 1—Small localized tumor, no MYCN amplification or chromothripsis. | Surgery, chemotherapy | >95% |
| Stage 2—Localized tumor, some lymph node involvement, no MYCN amplification or chromothripsis. | Surgery, chemotherapy | ||||
| 4S |  |
Stage 4s—Localized primary tumor, metastasis to liver, skin, bone marrow; diagnosed in infants <12 months of age. No MYCN amplification. | Observation | ||
| Intermediate | 3–4 |  |
Stage 3—Tumor infiltrating across midline, regional or contralateral lymph node involvement, no MYCN amplification. | Surgery, chemotherapy | 90%–95% |
| Stage 4—Primary tumor, metastasis to lymph nodes, bone marrow, bone, skin, liver; diagnosed <12 months of age, no MYCN amplification. | Surgery, chemotherapy | ||||
| High | 3–4 |  |
Stage 3—Tumor infiltrating across midline, regional or contralateral lymph node involvement, MYCN amplified. | Surgery, chemotherapy, radiotherapy, high-dose chemotherapy with autologous stem cell rescue, biologic and immunotherapeutic maintenance therapy, retinoids | 40%–50% |
| Stage 4—Primary tumor, metastasis to lymph nodes, bone marrow, bone, skin, liver; diagnosed >12 months of age, MYCN amplified. |