Sandbox:staging

Neuroblastoma Staging

International Neuroblastoma Staging System (INSS)

The staging of neuroblastoma is based on the International Neuroblastoma Staging System (INSS).

T	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)

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 4S should be minimal (<10% of total nucleated cells identified as malignant on bone marrow biopsy)

Risk Stratification

• Neuroblastoma patients are risk stratified into a low risk group, an intermediate risk group, and a high risk group based on the tumor INSS stage, tumor grade the patient's age, and the molecular pathway involved.

• Low risk neuroblastoma groups include:

• Patients with stage 1 tumor irrespective of the age, grade, and molecular pathway.
• Patients with stage 2A/2B, irrespective of age, irrespective of grade, MYCN-NA molecular pathway
• Patients with Stage 4S, <365 days of age, favorable histology, DNA Index >1 , MYCN-NA molecular pathway

• Intermediate risk neuroblastoma groups include:

• Patients with stage 1 tumor irrespective of the age, grade, and molecular pathway.

Group 2

2A/2B (<50% resected or Bx only) 0–12 years MYCN-NA, any histology/ploidy* 3 <365 days MYCN-NA, FH, DI>1* 3 ≥365 days - 12 years MYCN-NA, FH* 4S (symptomatic) <365 days MYCN-NA, FH, DI>1*

 Group 3

3 <365 days MYCN-NA, either UH or DI=1* 4 <365 days MYCN-NA, FH, DI>1* 4S <365 days MYCN-NA, either UH or DI=1*; or unknown biology

 Group 4

4 <365 days MYCN-NA, either DI=1 or UH 3 365 -<547 days MYCN-NA, UH, any ploidy 4 365 -<547 days MYCN-NA, FH, DI>1

• High risk neuroblastoma groups include:

2A/2B, 3, 4, 4S Any MYCN-amplified, any histology/ploidy 3 ≥ 547 days MYCN-NA, UH, any ploidy 4 365 - >547 days MYCN-NA, UH or DI=1 4 > 547 days Any