Adrenal gland and paraganglia
Neuroblastic tumors
Staging of neuroblastic tumors

Author: Debra Zynger, M.D. (see Authors page)

Revised: 29 January 2018, last major update January 2018

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Staging of neuroblastic tumors

Cite this page: Zynger, D. Staging of neuroblastic tumors. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/adrenalstagingneuroblastic.html. Accessed June 19th, 2018.
International Neuroblastoma Staging System (INSS)
  • 1: localized tumor with complete gross (not necessarily microscopic) excision, negative representative ipsilateral lymph nodes microscopically; note: nodes removed with primary tumor may be positive; includes grossly resectable tumor in midline from pelvic ganglia or organ of Zuckerkandl
  • 2A: localized tumor with incomplete gross excision, negative representative nonadherent ipsilateral lymph nodes microscopically; note: includes midline tumor that extends beyond one side of vertebral column and is unresectable
  • 2B: localized tumor with positive representative nonadherent ipsilateral lymph nodes; enlarged contralateral lymph nodes must be negative microscopically; note: includes midline tumor that extends beyond one side of vertebral column, is unresectable with positive ipsilateral lymph node involvement (on side of extension); also includes a thoracic tumor with malignant unilateral pleural effusion
  • 3: unresectable unilateral tumor infiltrating across midline or localized unilateral tumor with contralateral regional lymph node involvement; note: includes midline tumor with bilateral extension by infiltration (unresectable) or by lymph node involvement; includes a tumor of any size with malignant ascites or peritoneal implants
  • 4: any primary tumor with dissemination to distant lymph nodes, bone, bone marrow, liver, skin or other organs (except 4S)
  • 4S: localized primary tumor (stage 1, 2A or 2B) with dissemination limited to skin, liver or bone marrow (limited to infants < 1 year of age); note: marrow involvement should be minimal (< 10% of total nucleated cell identified as malignant); more extensive involvement should be classified as stage 4
  • Note: for infants < 1 year, stages 1, 2A, 2B, 3 and 4S have similar rates of 4 year overall survival (98.5%) compared to stage 4 (73%)
International Neuroblastoma Pathology Classification (INPC) histology group
Favorable histopathology
  • Any age
    • Ganglioneuroblastoma, intermixed
    • Ganglioneuroma
  • < 18 months
    • Neuroblastoma, poorly differentiated subtype with low or intermediate mitosis-karyorrhexis index
    • Neuroblastoma, differentiating subtype with low or intermediate mitosis-karyorrhexis index
  • 18 - 60 months
    • Neuroblastoma, differentiating subtype with low mitosis-karyorrhexis index

Unfavorable histopathology
  • Any age
    • Neuroblastoma, undifferentiated subtype
    • Neuroblastoma, any subtype with high mitosis-karyorrhexis index
  • 18 - 60 months
    • Neuroblastoma, poorly differentiated subtype
    • Neuroblastoma, differentiating subtype with intermediate mitosis-karyorrhexis index
  • > 60 months
    • Neuroblastoma, any subtype
Diagrams / tables

Images hosted on other servers:

Children's Oncology
Group (COG)
neuroblastoma risk
grouping system

INRG stage definitions

INRG consensus pretreatment classification