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 imaged-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
- stage MS
More than 20 imaged-defined risk factors (IDRF) have been extensively defined by the INRG 5,6, and these are outside the scope of this article.
- 1. Brodeur GM, Pritchard J, Berthold F et-al. Revisions of the international criteria for neuroblastoma diagnosis, staging, and response to treatment. J. Clin. Oncol. 1993;11 (8): 1466-77. J. Clin. Oncol. (abstract) - Pubmed citation
- 2. Kumar V, Abbas AK, Fausto N. Robbins and Cotran pathologic basis of disease. W B Saunders Co. (2005) ISBN:0721601871. Read it at Google Books - Find it at Amazon
- 3. Lonergan Gael J., Cornelia M. Schwab, Eric S. Suarez and Christian L. Carlson. “From the Archives of the AFIP.” Radiographics 22, no. 4 (July 2002): 911-934. doi:VL - 22.
- 4. Kushner BH. Neuroblastoma: a disease requiring a multitude of imaging studies. J. Nucl. Med. 2004;45 (7): 1172-88. J. Nucl. Med. (full text) - Pubmed citation
- 5. McCarville MB. Imaging neuroblastoma: what the radiologist needs to know. Cancer Imaging. 2011;11 Spec No A (1A): S44-7. doi:10.1102/1470-7330.2011.9008 - Free text at pubmed - Pubmed citation
- 6. Monclair T, Brodeur GM, Ambros PF et-al. The International Neuroblastoma Risk Group (INRG) staging system: an INRG Task Force report. J. Clin. Oncol. 2009;27 (2): 298-303. doi:10.1200/JCO.2008.16.6876 - Free text at pubmed - Pubmed citation