Neuroblastoma (staging)

Last revised by Abenezer Zinaye on 18 Mar 2021

There are two methods of neuroblastoma staging, one that is based on post-operative patients (INSS) and one developed for pre-treatment patients (INRGSS).


International Neuroblastoma Staging System (INSS)

This staging system is for post-operative patients and mainly for prognosis 1:

  • stage 1
    • localized tumor with complete gross excision with or without microscopic residual disease
    • contralateral and representative ipsilateral regional lymph nodes negative for disease (nodes attached to and removed with primary tumor may be positive)
  • stage 2a
    • localized tumor with incomplete gross excision
    • ipsilateral and contralateral nodes negative for tumor
  • stage 2b
    • localized tumor with complete or incomplete resection
    • positive ipsilateral (non-adherent) nodes
    • contralateral nodes negative for tumor
  • stage 3
    • unresectable lateral tumor that crosses the midline or
    • localized tumor with contralateral regional lymph node involvement
    • midline tumor with bilateral extension by infiltration or by lymph node involvement
  • stage 4S ("special")
    • <1 year of age
    • localized tumor (stage 1, 2A or 2B)
    • distant metastases confined to skin, liver and/or bone marrow
  • stage 4
    • distant metastases not fulfilling stage 4S

In the majority of cases (60-80%), patients present with stage 3 or 4 tumors 2.

International Neuroblastoma Risk Group Staging System (INRGSS)

The INRG was developed after INSS to formulate staging for pre-operative neuroblastoma 5,6:

  • stage L1
    • localized tumors confined to one body cavity and not involving image-defined risk factors (IDRFs)
  • stage L2
    • locoregional tumors involving one or more IDRFs
  • stage M
    • distant (remote) metastases (i.e. excludes metastases to local lymph node groups)
    • excludes stage MS
  • stage MS
    • metastases in patients <18 months (some centers <12 months) confined to skin, liver and/or bone marrow

More than 20 image-defined risk factors (IDRF) have been extensively defined by the INRG 5,6, and these are outside the scope of this article.

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