Pathologic Staging
CAP Approved Pediatric • Neuroblastoma 3.0.0.1
International Neuroblastoma Staging System (INSS)#
___ Stage 1
• localized tumor with complete gross excision, with or without microscopic
residual disease
• representative ipsilateral nonadherent lymph nodes negative for tumor
microscopically (nodes attached to and removed with the primary tumor may
be positive)
___ Stage 2A
• localized tumor with incomplete gross excision; representative ipsilateral
nonadherent lymph nodes negative for tumor microscopically
___ Stage 2B
• localized tumor with or without complete gross excision with ipsilateral
nonadherent lymph nodes positive for tumor; enlarged contralateral lymph
nodes must be negative microscopically
___ Stage 3
• unresectable unilateral tumor infiltrating across the midline,## with or
without regional lymph node involvement
• localized unilateral tumor with contralateral regional lymph node
involvement
• midline tumor with bilateral extension by infiltration (unresectable) or by
lymph node involvement
___ Stage 4
• any primary tumor with dissemination to distant lymph nodes, bone, bone
marrow, liver, skin, and/or other organs (except as defined for stage 4S###)
___ Stage 4S
• localized primary tumor (as defined for stage 1, 2A, or 2B), with
dissemination limited to skin, liver, and/or bone marrow### (limited to
infants less than 1 year old)
# Multifocal primary tumors (eg, bilateral adrenal primary tumors) should be staged according to the greatest extent of disease, as defined above, and followed by a subscript “M” (eg, 3M).
## 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.
### Marrow involvement in stage 4S should be minimal (ie, less than 10% of total nucleated cells identified as malignant on bone marrow biopsy or marrow aspirate). More extensive marrow involvement would be considered stage 4. The meta-iodobenzylguanidine (MIBG) scan (if performed) should be negative in the marrow.