The International Neuroblastoma Staging System (INSS) distinguishes between the following disease stages:
| Stage | Indicator |
| 1 | Localized tumor confined to site of originor tumor completely removed |
| 2a | Localized tumor infiltrates surrounding area without crossing midline, no lymph node involvementor Tumor infestation not completely removed only on one side of the spineNo lymph node involvement in the vicinity of the tumor |
| 2b | Localized tumor infiltrates surrounding area without crossing midline, homolateral lymph node involvement or Completely or incompletely removed tumorinvolvement only on one side of spineadjacent lymph nodes on same side of body are involved |
| 3 | Localized tumor crosses midline, regional lymph nodes may be affected on both sidesbtw. Tumor not completely removed with spinal crossing or involvement of lymph nodes on opposite side of body from tumor |
| 4 | Hematogenous distant metastases |
| 4S | In infancy only (age limit by newer criteria to 18 months)Metastases only to skin, liver, and/or, to a minimal extent, bone marrowNote: May regress spontaneously, so may wait if infant is clinically stable and monitored carefully. |
