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===International Neuroblastoma Risk Group Staging System (INRGSS)===
===International Neuroblastoma Risk Group Staging System (INRGSS)===
According to the newly proposed International Neuroblastoma Risk Group Staging System (INRGSS), there are four stages of neuroblastoma based on the extent of dissemination and image findings.<ref name="wiki">Neuroblastoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 5 2015</ref>


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==Risk Stratification==
==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.
* 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, the patient's age, tumor grade, and the amplification of ''MYCN'' gene.<ref name="pmid22248965">{{cite journal| author=Davidoff AM| title=Neuroblastoma. | journal=Semin Pediatr Surg | year= 2012 | volume= 21 | issue= 1 | pages= 2-14 | pmid=22248965 | doi=10.1053/j.sempedsurg.2011.10.009 | pmc=PMC3261589 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22248965  }} </ref>
 
* '''Low risk neuroblastoma groups''' include:
* '''Low risk neuroblastoma groups''' include:
:* Patients with stage 1 tumor irrespective of the age, grade, and molecular pathway.
:* 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 2A/2B (>50% resected), irrespective of age, irrespective of the grade, ''MYCN''not amplified
:* Patients with Stage 4S, <365 days of age, favorable histology, DNA Index >1 , MYCN-NA molecular pathway
:* Patients with Stage 4S, <365 days of age, favorable histology, DNA index >1 , ''MYCN'' not amplified


* '''Intermediate risk neuroblastoma groups''' include:
* '''Intermediate risk neuroblastoma groups''' include:
:* Patients with stage 1 tumor irrespective of the age, grade, and molecular pathway.
:* '''Group 1'''
Group 2
::* Patients with stage 2A/2B (<50% resected or Bx only) tumor, 0-12 years of age, irrespective of the grade, ''MYCN''not amplified
 
::* Patients with stage 3 tumor, <365 days of age, favorable histology, DNA index >1, ''MYCN''not amplified
2A/2B (<50% resected or Bx only)
::* Patients with stage 3 tumor, 1-12 years of age, favorable histology, ''MYCN''not amplified
0–12 years
::* Patients with symptomatic stage 4S, <365 days of age, favorable histology, DNA index >1, ''MYCN''not amplified
MYCN-NA, any histology/ploidy*
:* '''Group 2'''
3
::* Patients with stage 3 tumor, <365 days of age, unfavorable histology, DNA index=1, ''MYCN''not amplified
<365 days
::* Patients with stage 4 tumor, <365 days of age, favorable histology, DNA index >1, ''MYCN'' not amplified
MYCN-NA, FH, DI>1*
::* Patients with stage 4S tumor, <365 days of age, unfavorable histology, DNA index=1, ''MYCN'' not amplified
3
:* '''Group 3'''
≥365 days - 12 years
::* Patients with stage 4 tumor, <365 days of age, unfavorable histology, DNA index=1, ''MYCN'' not amplified
MYCN-NA, FH*
::* Patients with stage 3 tumor, 365-547 days of age, unfavorable histology, ''MYCN'' not amplified
4S (symptomatic)
::* Patients with stage 4 tumor, 365-547 days of age, favorable histology, DNA index >1, ''MYCN'' not amplified
<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:
* '''High risk neuroblastoma groups''' include:
 
:* Patients with stage 2A/2B, 3, 4, or 4S tumor, irrespective of the age, irrespective of the grade, ''MYCN'' amplified
 
:* Patients with stage 3 tumor, ≥ 547 days of age, unfavorable histology, ''MYCN'' not amplified
2A/2B, 3, 4, 4S
:* Patients with stage 4 tumor, 365-547 days of age, unfavorable histology, DNA index=1, ''MYCN'' not amplified
Any
:* Patients with stage 4 tumor, >547 days of age, irrespective the grade and ''MYCN'' amplification state
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

Latest revision as of 17:00, 7 October 2015

Neuroblastoma Staging

International Neuroblastoma Staging System (INSS)

The staging of neuroblastoma is based on the International Neuroblastoma Staging System (INSS).[1][2]

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)

According to the newly proposed International Neuroblastoma Risk Group Staging System (INRGSS), there are four stages of neuroblastoma based on the extent of dissemination and image findings.[1]

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, the patient's age, tumor grade, and the amplification of MYCN gene.[2]
  • Low risk neuroblastoma groups include:
  • Patients with stage 1 tumor irrespective of the age, grade, and molecular pathway
  • Patients with stage 2A/2B (>50% resected), irrespective of age, irrespective of the grade, MYCNnot amplified
  • Patients with Stage 4S, <365 days of age, favorable histology, DNA index >1 , MYCN not amplified
  • Intermediate risk neuroblastoma groups include:
  • Group 1
  • Patients with stage 2A/2B (<50% resected or Bx only) tumor, 0-12 years of age, irrespective of the grade, MYCNnot amplified
  • Patients with stage 3 tumor, <365 days of age, favorable histology, DNA index >1, MYCNnot amplified
  • Patients with stage 3 tumor, 1-12 years of age, favorable histology, MYCNnot amplified
  • Patients with symptomatic stage 4S, <365 days of age, favorable histology, DNA index >1, MYCNnot amplified
  • Group 2
  • Patients with stage 3 tumor, <365 days of age, unfavorable histology, DNA index=1, MYCNnot amplified
  • Patients with stage 4 tumor, <365 days of age, favorable histology, DNA index >1, MYCN not amplified
  • Patients with stage 4S tumor, <365 days of age, unfavorable histology, DNA index=1, MYCN not amplified
  • Group 3
  • Patients with stage 4 tumor, <365 days of age, unfavorable histology, DNA index=1, MYCN not amplified
  • Patients with stage 3 tumor, 365-547 days of age, unfavorable histology, MYCN not amplified
  • Patients with stage 4 tumor, 365-547 days of age, favorable histology, DNA index >1, MYCN not amplified
  • High risk neuroblastoma groups include:
  • Patients with stage 2A/2B, 3, 4, or 4S tumor, irrespective of the age, irrespective of the grade, MYCN amplified
  • Patients with stage 3 tumor, ≥ 547 days of age, unfavorable histology, MYCN not amplified
  • Patients with stage 4 tumor, 365-547 days of age, unfavorable histology, DNA index=1, MYCN not amplified
  • Patients with stage 4 tumor, >547 days of age, irrespective the grade and MYCN amplification state
  1. ↑ 1.0 1.1 Neuroblastoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 5 2015
  2. ↑ 2.0 2.1 Davidoff AM (2012). "Neuroblastoma". Semin Pediatr Surg. 21 (1): 2–14. doi:10.1053/j.sempedsurg.2011.10.009. PMC 3261589. PMID 22248965.
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