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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:
- 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)
- localized tumor with incomplete gross excision
- ipsilateral and contralateral nodes negative for tumor
- localized tumor with complete or incomplete resection
- positive ipsilateral (non-adherent) nodes
- contralateral nodes negative for tumor
- 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")
- 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:
- localized tumors confined to one body cavity and not involving image-defined risk factors (IDRFs)
- locoregional tumors involving one or more IDRFs
- distant (remote) metastases (i.e. excludes metastases to local lymph node groups)
- excludes 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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