Neuroblastoma staging

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2] Zahir Ali Shaikh, MD[3]

Overview

According to the conventional International Neuroblastoma Staging System (INSS), there are 6 stages of neuroblastoma based on the tumor size, lymph node involvement, and presence of metastasis. However, according to a newly proposed International Neuroblastoma Risk Group Staging System (INRGSS), there are four stages of neuroblastoma based on the extent of dissemination and image findings. Neuroblastoma patients are risk stratified according to the Children Oncology Group (COG) risk stratification system into a low risk group, an intermediate risk group, and a high risk group based on the tumor INSS stage, the patient's age, tumor grade, and the amplification of MYCN gene.

Neuroblastoma Staging

International Neuroblastoma Staging System (INSS)

• According to the conventional International Neuroblastoma Staging System (INSS), there are 6 stages of neuroblastoma based on the tumor size, lymph node involvement, and presence of metastasis.

Stage	Description
Stage 1	Localized tumor Complete gross excision Ipsilateral lymph nodes involvement negative under microscopic examination
Stage 2A	Localized tumor Incomplete gross resection Ipsilateral lymph nodes involvement negative under microscopic examination
Stage 2B	Localized tumor Complete or incomplete gross resection Ipsilateral lymph nodes involvement positive under microscopic examination Enlarged contralateral lymph nodes but with negative involvement under microscopic examination
Stage 3	Localized unilateral tumor with contralateral lymph nodes involvement positive under microscopic examination or Unresectable unilateral tumor infiltrating across the midline with positive or negative regional lymph node involvement or Unresectable midline tumor with bilateral infiltration or lymph node involvement
Stage 4	Metastasis of the tumor to distant lymph nodes Metastasis of the tumor to liver, skin, bone and/or other organs (except as defined by Stage 4S)
Stage 4S	Limited to infants <1 year of age Localized primary tumor (as defined for stage 1, 2A, or 2B) Metastasis of the tumor limited to skin, liver and/or bone marrow Bone marrow involvement in stage 4S should be minimal (<10% of total nucleated cells identified as malignant on bone marrow biopsy)

International Neuroblastoma Risk Group Staging System (INRGSS)

According to a newly proposed International Neuroblastoma Risk Group Staging System (INRGSS), there are four stages of neuroblastoma based on the extent of dissemination and image findings.

Stage	Description
Stage L1	Localized disease without image-defined risk factors
Stage L2	Localized disease with image-defined risk factors
Stage M	Metastatic disease
Stage MS	Metastatic disease limited to skin, liver and/or bone marrow Bone marrow involvement in stage MS should be minimal (<10% of total nucleated cells identified as malignant on bone marrow biopsy)

Risk Stratification

Children's Oncology Group Risk Stratification

• Children Oncology Group (COG) risk stratification system determines the protocol of management used for neuroblastoma patients.
• Neuroblastoma patients are risk stratified into a low risk group, intermediate risk groups, and a high risk group based on the INSS stage, the patient's age, histological grade, and the amplification of MYCN gene.

INSS stage 1 Irrespective of age Irrespective of grade Irrespective of molecular pathway

Low risk neuroblastoma patients (group 1)

INSS stage 2A/2B (>50% resected) Irrespective of age Irrespective of grade MYCN not amplified

INNS stage 4S <365 days of age Favorable histology DNA index >1 MYCN not amplified

INNS stage 2A/2B (<50% resected) 0-12 years of age Irrespective of grade MYCN not amplified

INSS stage 3 <365 days of age Favorable histology DNA index >1 MYCN not amplified

Intermediate risk neuroblastoma patients (group 2)

INNS stage 3 1-12 years of age Favorable histology MYCN not amplified

Symptomatic INNS stage 4S <365 days of age Favorable histology DNA index >1 MYCN not amplified

INSS stage 3 <365 days of age Unfavorable histology DNA index=1 MYCN not amplified

Intermediate risk neuroblastoma patients (group 3)

INSS stage 4 <365 days of age Favorable histology DNA index >1 MYCN not amplified

INNS stage 4S <365 days of age Unfavorable histology DNA index=1 MYCN not amplified

INSS stage 4 <365 days of age Unfavorable histology DNA index=1 MYCN not amplified

Intermediate risk neuroblastoma patients (group 4)

INSS stage 3 365-547 days of age Unfavorable histology MYCN not amplified

INNS stage 4 365-547 days of age Favorable histology DNA index>1 MYCN not amplified

INNS stage 2A/2B, Stage 3 , Stage 4 or Stage 4S Irrespective of age Irrespective of grade MYCN amplified

INSS stage 3 >547 days of age Unfavorable histology MYCN not amplified

High risk neuroblastoma patients (group 5)

INNS stage 4 365-547 days of age Unfavorable histology DNA index=1 MYCN not amplified

INNS stage 4 >547 days of age Irrespective grade Irrespective MYCN amplification state

References

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