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Neuroblastoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Stage Information for Neuroblastoma

Table 3. The International Neuroblastoma Staging System (INSS)

Stage/Prognostic GroupDescription
mIBG = metaiodobenzylguanidine.
Stage 1Localized tumor with complete gross excision, with or without microscopic residual disease; representative ipsilateral lymph nodes negative for tumor microscopically (i.e., nodes attached to and removed with the primary tumor may be positive).
Stage 2ALocalized tumor with incomplete gross excision; representative ipsilateral nonadherent lymph nodes negative for tumor microscopically.
Stage 2BLocalized tumor with or without complete gross excision, with ipsilateral nonadherent lymph nodes positive for tumor. Enlarged contralateral lymph nodes must be negative microscopically
Stage 3Unresectable unilateral tumor infiltrating across the midline, with or without regional lymph node involvement; or localized unilateral tumor with contralateral regional lymph node involvement; or midline tumor with bilateral extension by infiltration (unresectable) or by lymph node involvement. The midline is defined as the vertebral column. Tumors originating on one side and crossing the midline must infiltrate to or beyond the opposite side of the vertebral column.
Stage 4Any primary tumor with dissemination to distant lymph nodes, bone, bone marrow, liver, skin, and/or other organs, except as defined for stage 4S.
Stage 4SLocalized primary tumor, as defined for stage 1, 2A, or 2B, with dissemination limited to skin, liver, and/or bone marrow (by definition limited to infants younger than 12 months).[3]Marrow involvement should be minimal (i.e., <10% of total nucleated cells identified as malignant by bone biopsy or by bone marrow aspirate). More extensive bone marrow involvement would be considered stage 4 disease. The results of the mIBG scan, if performed, should be negative for disease in the bone marrow.

Controversy exists regarding the INSS staging system and the treatment of several small subsets of patients.[16,17,18] Risk group assignment and recommended treatment are expected to evolve as additional outcome data are analyzed. For example, the risk group for INSS stage 4, including patients aged 12 to 18 months, changed in 2005 for patients with MYCN-nonamplified status.[19,20,21]

International Neuroblastoma Risk Group Staging System (INRGSS)

The INRGSS is a preoperative staging system that was developed specifically for the INRG cellular classification system. The extent of disease is determined by the presence or absence of image-defined risk factors (IDRFs) and/or metastatic tumor at the time of diagnosis, before any treatment. IDRFs are surgical risk factors, detected by imaging, that make total tumor excision risky or difficult at the time of diagnosis.

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