Adrenal gland and paraganglia
Neuroblastic tumors
Staging of neuroblastic tumors

Author: Nat Pernick, M.D. (see Authors page)

Revised: 25 January 2016, last major update February 2005

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

Staging / Staging classifications

Evans staging of neuroblastomas
  • I: tumor confined to structure or organ of origin
  • II: tumor extends in continuity beyond structure or organ of origin, doesn't cross midline and variable ipsilateral nodal metastases
  • III: tumor extends in continuity beyond midline, variable bilateral nodal metastases
  • IV: tumor metastatic to viscera, distal lymph nodes, soft tissue and skeleton
  • IV-S (special): stage I or II with remote disease in liver, skin and bone marrow (with no bony destruction)

  • Stage IV-S (4S): small / undetectable primaries with disease involving liver, skin or bone marrow; survival of 60-90%; median age 4 months, primary usually adrenal (also retroperitoneum, mediastinum); usually have favorable histology; patients dying of progressive disease have either unfavorable histology or N-myc amplification (Hum Pathol 1996;27:1135)

  • Ten year survival by Evans stage: I-88%, II-90%, III-63%; IV-21% and IVs-81%


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 non-adherent 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 non-adherent 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%)