Staging (Neuroblastoma)                                                
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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.


Pediatric


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