How is pediatric neuroblastoma staged?

Updated: Oct 09, 2017
  • Author: Norman J Lacayo, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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The patient should undergo a staging workup along with surgical resection or biopsy, as appropriate. Using various molecular features in conjunction with pathology and staging is essential to appropriately stratify patients and determine the best therapy.

The International Neuroblastoma Staging System (INSS) is currently used in all cooperative group studies in the United States. Recently, the International Neuroblastoma Risk Group Staging System (INRGSS) and International Neuroblastoma Risk Group Consensus Pretreatment Classification were released. [16] The current INSS system is based on degree of surgical resection and thus is not appropriate for use with the INRG Pretreatment Classification. This is especially important because not all groups use upfront surgical resection as part of their staging system. The INRG was formulated to be used in international settings and to facilitate comparison of treatment outcomes across studies to allow common definitions among all groups. Thus, development of the INRGSS was facilitated using pretreatment tumor imaging rather than extent of surgical resection.

The INRGSS is as follows:

  • L1 - Localized tumor not involving vital structures, as defined by the list of image-defined risk factors and confined to one body component

  • L2 - Locoregional tumor with presence of one or more image-defined risk factors

  • M - Distant metastatic disease

  • MS - Metastatic disease in children younger than 18 months with metastases confined to skin, liver, and/or bone marrow

The INSS is as follows:

  • Stage 1

    • Localized tumor with complete gross excision, microscopic residual disease, or both

    • Ipsilateral lymph nodes negative for tumor (Nodes attached to the primary tumor may be positive for tumor).

  • Stage 2A

    • Localized tumor with incomplete gross resection

    • Representative ipsilateral nonadherent lymph nodes microscopically negative for tumor

  • Stage 2B

    • Localized tumor, complete gross excision, or both with ipsilateral nonadherent lymph nodes positive for tumor

    • Enlarged contralateral lymph nodes, which are negative for tumor microscopically

  • Stage 3

    • Unresectable unilateral tumor infiltrating across the midline, regional lymph node involvement, or both

    • Alternatively, localized unilateral tumor with contralateral regional lymph node involvement

  • Stage 4 - Any primary tumor with dissemination to distant lymph nodes, bone, bone marrow, liver, skin, and/or other organs (except as defined for stage 4S)

  • Stage 4S

    • Localized primary tumor (as defined for stages 1, 2A, or 2B) with dissemination limited to skin, liver, and/or bone marrow (< 10% involvement)

    • Limited to infants

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