Neuroblastoma (staging)

Last revised by Ciléin Kearns on 15 May 2024

There are two methods of neuroblastoma staging; the International Neuroblastoma Risk Group Staging System (INRGSS, based on imaging of pre-treatment patients), and the International Neuroblastoma Staging System (INSS, based on the outcomes of surgery).

International Neuroblastoma Risk Group Staging System (INRGSS)

The International Neuroblastoma Risk Group (INRGSS) was developed after INSS for 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 defined by the INRG 5,6, and these are outside the scope of this article.

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.

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