Staging

Table 18-5 shows a comparison of two staging systems—INSS and Children's Oncology Group (COG)-for neuroblastoma that are currently in use. Table 18-6 shows the modified International Neuroblastoma Response Criteria (INRC) definitions of response to treatment.

Table 18-5. Comparison of Staging Systems (INSS and COG) for Neuroblastoma

INSS system (International Staging System)

COG system (Children's Oncology Group)

Stage 1. Localized tumor with complete gross excision, with or without microscopic residual disease; representative ipsilateral lymph nodes negative for tumor microscopically (nodes attached to and removed with the primary tumor may be positive).

Stage 2A. Localized tumor with incomplete gross excision; representative ipsilateral nonadherent lymph nodes negative for tumor microscopically.

Stage 2B. Localized tumor with or without complete gross excision, with ipsilateral nonadherent lymph nodes positive for tumor. Enlarged contralateral lymph nodes must be negative microscopically.

Stage 3. Unresectable unilateral tumor infiltrating across the midline,a with or without regional lymph node

Stage 1. Tumor confined to organ or structure of origin.

Stage 2. Tumor extending in continuity beyond the organ or structure of origin, but not crossing the midline. Regional lymph nodes on the ipsilateral side may be involved.

Stage 3. Tumor extending in continuity beyond the midline. Regional lymph nodes may be involved bilaterally.

Table 18-5. (Continued)

INSS system (International Staging System) COG system (Children's Oncology Group)

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. 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 stage 1, 2A, or 2B), with dissemination limited to skin, liver, and/or bone marrow6 (limited to infants <1 yr of age).

Multifocal primary tumors (e.g., bilateral adrenal primary tumors) should be staged according to the greatest extent of disease, as defined above, and followed by a subscript letter M (e.g., 3M).

aThe 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.

A grossly resectable tumor arising in the midline from pelvic ganglia or the organ of Zuckerkandl is stage 1. A midline tumor extending beyond one side of the vertebral column that is unresectable is stage 2A. Ipsilateral lymph node involvement is stage 2B, whereas bilateral lymph node involvement is stage 3. A midline primary tumor with bilateral infiltration that was not resectable is stage 3. A tumor of any size with malignant ascites or peritoneal implants is stage 3 (but a thoracic tumor with malignant pleural effusion unilaterally is stage 2B).

•"Marrow involvement in stage 4S should be minimal, i.e., <10% of total nucleated cells identified as malignant on bone marrow biopsy or on marrow aspirate. More extensive marrow involvement is stage 4. The MIBG scan should be negative in the marrow.

Table 18-6. Modified International Neuroblastoma Response Criteria (INRC)

Definitions of Response to Treatment

Table 18-6. Modified International Neuroblastoma Response Criteria (INRC)

Response

Primary tumor0

Metastatic sites"'6

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