Neuroblastoma staging: Difference between revisions

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{{Neuroblastoma}}
{{Neuroblastoma}}
{{CMG}}
{{CMG}} {{AE}}{{HL}}{{ZAS}}


==Overview==
==Overview==
Diagnosis of neuroblastoma can be complicated. It has been called the "great masquerader" because its symptoms mimic so many other diseases. Even a pathological study (biopsy) might reveal cells that can resemble other small round blue tumor cells, like[[lymphoma|lymphomas]] and [[rhabdomyosarcoma|rhabdomyosarcomas]]. Only a pathologist familiar with neuroblastoma can distinguish the difference (and neuroblastoma is rare).
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|patient's]] [[age]], [[tumor]] grade, and the amplification of ''MYCN'' [[gene]].
==Staging ==
Neuroblastoma is stratified according to its anatomical presence at diagnosis:


*Stage 1: Localized tumor confined to the area of origin.
==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]].<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><ref name="pmid21736987">{{cite journal| author=Colon NC, Chung DH| title=Neuroblastoma. | journal=Adv Pediatr | year= 2011 | volume= 58 | issue= 1 | pages= 297-311 | pmid=21736987 | doi=10.1016/j.yapd.2011.03.011 | pmc=PMC3668791 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21736987  }} </ref><ref>{{Cite journal
| author = [[H. Shimada]], [[S. Umehara]], [[Y. Monobe]], [[Y. Hachitanda]], [[A. Nakagawa]], [[S. Goto]], [[R. B. Gerbing]], [[D. O. Stram]], [[J. N. Lukens]] & [[K. K. Matthay]]
| title = International neuroblastoma pathology classification for prognostic evaluation of patients with peripheral neuroblastic tumors: a report from the Children's Cancer Group
| journal = [[Cancer]]
| volume = 92
| issue = 9
| pages = 2451–2461
| year = 2001
| month = November
| pmid = 11745303
}}</ref><ref>{{Cite journal
| author = [[D. de Sa]]
| title = Neuroblastoma classification
| journal = [[Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society]]
| volume = 3
| issue = 5
| pages = 506–507
| year = 2000
| month = September-October
| pmid = 10890940
}}</ref><ref>{{Cite journal
| author = [[S. Goto]], [[S. Umehara]], [[R. B. Gerbing]], [[D. O. Stram]], [[G. M. Brodeur]], [[R. C. Seeger]], [[J. N. Lukens]], [[K. K. Matthay]] & [[H. Shimada]]
| title = Histopathology (International Neuroblastoma Pathology Classification) and MYCN status in patients with peripheral neuroblastic tumors: a report from the Children's Cancer Group
| journal = [[Cancer]]
| volume = 92
| issue = 10
| pages = 2699–2708
| year = 2001
| month = November
| pmid = 11745206
}}</ref>


*Stage 2A: Unilateral tumor with incomplete gross resection; identifiable ipsilateral and contralateral lymph node negative for tumor.
{| style="cellpadding=0; cellspacing= 0; width: 950px;"
|-
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''Stage'''|| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="center" |'''Description'''
|-
| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" |'''Stage 1''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
* Localized [[tumor]]
* Complete gross [[excision]]
* [[Ipsilateral]] [[Lymph node|lymph nodes]] involvement negative under [[microscopic examination]]
|-


*Stage 2B: Unilateral tumor with complete or incomplete gross resection; with ispilateral lymph node positive for tumor; identifiable contralateral lymph node negative for tumor.
| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" |'''Stage 2A''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
   
* Localized [[tumor]]
*Stage 3: Tumor infiltrating across midline with or without regional lymph node involvement; or unilateral tumor with contralateral lymph node involvement; or midline tumor with bilateral lymph node involvement.
* Incomplete gross [[resection]]
* [[Ipsilateral]] [[Lymph node|lymph nodes]] involvement negative under [[microscopic examination]]
|-


*Stage 4: Dissemination of tumor to distant lymph nodes, bone marrow, bone, liver, or other organs except as defined by Stage 4S.
| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" |'''Stage 2B''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
* Localized [[tumor]]
* Complete or incomplete gross [[resection]]
* [[Ipsilateral]] [[Lymph node|lymph nodes]] involvement positive under [[microscopic examination]]
* Enlarged [[contralateral]] [[Lymph node|lymph nodes]] but with negative involvement under [[microscopic examination]]


*Stage 4S: Localized primary tumor as defined in Stage 1 or 2, with dissemination limited to liver, skin, or bone marrow (less than 10 percent of nucleated bone marrow cells are tumors).
|-
 
| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" |'''Stage 3''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
* Localized unilateral [[tumor]] with [[contralateral]] [[Lymph node|lymph nodes]] involvement positive under [[microscopic examination]]
<center>'''or'''</center>
* Unresectable unilateral [[tumor]] infiltrating across the midline with positive or negative regional [[lymph node]] involvement
<center>'''or'''</center>
* Unresectable midline [[tumor]] with [[bilateral]] [[Infiltration (medical)|infiltration]] or [[lymph node]] involvement
 
|-
 
| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" |'''Stage 4''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
* [[Metastasis]] of the [[tumor]] to distant [[Lymph node|lymph nodes]]
* [[Metastasis]] of the [[tumor]] to [[liver]], [[skin]], [[bone]] and/or other [[organs]] (except as defined by Stage 4S)
 
|-
 
| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" |'''Stage 4S''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
* Limited to [[Infant|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.<ref name="pmid21736987">{{cite journal| author=Colon NC, Chung DH| title=Neuroblastoma. | journal=Adv Pediatr | year= 2011 | volume= 58 | issue= 1 | pages= 297-311 | pmid=21736987 | doi=10.1016/j.yapd.2011.03.011 | pmc=PMC3668791 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21736987  }} </ref><ref>{{Cite journal
| author = [[Susan L. Cohn]], [[Andrew D. J. Pearson]], [[Wendy B. London]], [[Tom Monclair]], [[Peter F. Ambros]], [[Garrett M. Brodeur]], [[Andreas Faldum]], [[Barbara Hero]], [[Tomoko Iehara]], [[David Machin]], [[Veronique Mosseri]], [[Thorsten Simon]], [[Alberto Garaventa]], [[Victoria Castel]] & [[Katherine K. Matthay]]
| title = The International Neuroblastoma Risk Group (INRG) classification system: an INRG Task Force report
| journal = [[Journal of clinical oncology : official journal of the American Society of Clinical Oncology]]
| volume = 27
| issue = 2
| pages = 289–297
| year = 2009
| month = January
| doi = 10.1200/JCO.2008.16.6785
| pmid = 19047291
}}</ref><ref>{{Cite journal
| author = [[Tom Monclair]], [[Garrett M. Brodeur]], [[Peter F. Ambros]], [[Herve J. Brisse]], [[Giovanni Cecchetto]], [[Keith Holmes]], [[Michio Kaneko]], [[Wendy B. London]], [[Katherine K. Matthay]], [[Jed G. Nuchtern]], [[Dietrich von Schweinitz]], [[Thorsten Simon]], [[Susan L. Cohn]] & [[Andrew D. J. Pearson]]
| title = The International Neuroblastoma Risk Group (INRG) staging system: an INRG Task Force report
| journal = [[Journal of clinical oncology : official journal of the American Society of Clinical Oncology]]
| volume = 27
| issue = 2
| pages = 298–303
| year = 2009
| month = January
| doi = 10.1200/JCO.2008.16.6876
| pmid = 19047290
}}</ref><ref>{{Cite journal
| author = [[Sidharth Mahapatra]] & [[Kishore B.. Challagundla]]
| title = Cancer, Neuroblastoma
| year = 2018
| month = January
| pmid = 28846355
}}</ref>
 
{| style="cellpadding=0; cellspacing= 0; width: 800px;"
|-
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''Stage'''|| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="center" |'''Description'''
|-
 
| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" |'''Stage L1'''|| style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
* Localized [[disease]] without image-defined [[Risk factor|risk factors]]
|-
 
| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" |'''Stage L2''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
* Localized [[disease]] with image-defined [[Risk factor|risk factors]]
 
|-
 
| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" |'''Stage M''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
* [[Metastatic disease]]
 
|-
 
| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" |'''Stage MS''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
* [[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 examination|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 [[Patient|patients]].<ref name="gov">Neuroblastoma treatment–for health professionals. National Cancer Institute (2015) http://www.cancer.gov/types/neuroblastoma/hp/neuroblastoma-treatment-pdq#section/_1 Accessed on October, 8 2015</ref>
* Neuroblastoma [[Patient|patients]] are risk stratified into a low risk group, intermediate risk groups, and a high risk group based on the INSS stage, the [[Patient|patient's]] [[age]], [[histological 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><ref name="pmid21736987">{{cite journal| author=Colon NC, Chung DH| title=Neuroblastoma. | journal=Adv Pediatr | year= 2011 | volume= 58 | issue= 1 | pages= 297-311 | pmid=21736987 | doi=10.1016/j.yapd.2011.03.011 | pmc=PMC3668791 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21736987  }} </ref><ref>{{Cite journal
| author = [[Nadja C. Colon]] & [[Dai H. Chung]]
| title = Neuroblastoma
| journal = [[Advances in pediatrics]]
| volume = 58
| issue = 1
| pages = 297–311
| year = 2011
| month =
| doi = 10.1016/j.yapd.2011.03.011
| pmid = 21736987
}}</ref>
<br>
{{familytree/start |summary=PE diagnosis Algorithm.}}
{{familytree | | | | | | | | | |,|-| A01 | | | | |A01=
<div style="width: 20em; padding:0em;text-align:left">
* '''INSS stage 1'''<br>
*'''Irrespective of [[age]]'''<br>
*'''Irrespective of grade'''<br>
*'''Irrespective of molecular pathway'''</div>}}
{{familytree | | | | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{familytree | | | | | | C01 |-|+|-| B01 | | | | |C01=<div style="width: 20em; padding:0em;text-align:center">'''Low risk neuroblastoma patients (group 1)'''</div>|B01=<div style="width: 20em; padding:0em;text-align:left">
*'''INSS stage 2A/2B (>50% resected)'''<br>
*'''Irrespective of [[age]]'''<br>
*'''Irrespective of grade'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree | | | | | | | | | |!| | | | | | | | | | | | | | | | | }}
{{familytree | | | | | | | | | |`|-| C02 | | | | | | |C02=<div style="width: 20em; padding:0em;text-align:left">
*'''INNS stage 4S'''<br>
*'''<365 days of [[age]]'''<br>
*'''Favorable [[histology]]'''<br>
*'''DNA index >1'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree/end}}
<br>
{{familytree/start |summary=PE diagnosis Algorithm.}}
{{familytree | | | | | | | | |,|-| A01 | | | | |A01=
<div style="width: 20em; padding:0em;text-align:left">
*'''INNS stage 2A/2B (<50% resected)'''<br>
*'''0-12 years of [[age]]'''<br>
*'''Irrespective of grade'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree | | | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{familytree | | | | | | | | |)|-| B01 | | | | ||B01=<div style="width: 20em; padding:0em;text-align:left">
*'''INSS stage 3'''<br>
*'''<365 days of [[age]]'''<br>
*'''Favorable [[histology]]'''<br>
*'''DNA index >1'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree | | | | | C01 |-|(| | | | | |C01=<div style="width: 20em; padding:1em;text-align:center">'''Intermediate risk neuroblastoma patients (group 2)'''</div> | | | | | | | | | | | | }}
{{familytree | | | | | | | | |)|-| C02 | | | | | | |C02=<div style="width: 20em; padding:0em;text-align:left">
*'''INNS stage 3'''<br>
*'''1-12 years of [[age]]'''<br>
*'''Favorable [[histology]]'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree | | | | | | | | |!| | | | | | | | | | | | | | | | | }}
{{familytree | | | | | | | | |`|-| C02 | | | | | | |C02=<div style="width: 20em; padding:0em;text-align:left">
*'''Symptomatic INNS stage 4S'''<br>
*'''<365 days of [[age]]'''<br>
*'''Favorable [[histology]]'''<br>
*'''DNA index >1'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree/end}}
<br>
{{familytree/start |summary=PE diagnosis Algorithm.}}
{{familytree | | | | | | | | | |,|-| A01 | | | | |A01=
<div style="width: 20em; padding:0em;text-align:left">
* '''INSS stage 3'''<br>
*'''<365 days of [[age]]'''<br>
*'''Unfavorable [[histology]]'''<br>
*'''DNA index=1'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree | | | | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{familytree | | | | | | C01 |-|+|-| B01 | | | | |C01=<div style="width: 20em; padding:0em;text-align:center">'''Intermediate risk neuroblastoma patients (group 3)'''</div>|B01=<div style="width: 20em; padding:0em;text-align:left">
*'''INSS stage 4'''<br>
*'''<365 days of [[age]]'''<br>
*'''Favorable [[histology]]'''<br>
*'''DNA index >1'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree | | | | | | | | | |!| | | | | | | | | | | | | | | | | }}
{{familytree | | | | | | | | | |`|-| C02 | | | | | | |C02=<div style="width: 20em; padding:0em;text-align:left">
*'''INNS stage 4S'''<br>
*'''<365 days of [[age]]'''<br>
*'''Unfavorable [[histology]]'''<br>
*'''DNA index=1'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree/end}}
<br>
{{familytree/start |summary=PE diagnosis Algorithm.}}
{{familytree | | | | | | | | | |,|-| A01 | | | | |A01=
<div style="width: 20em; padding:0em;text-align:left">
* '''INSS stage 4'''<br>
*'''<365 days of [[age]]'''<br>
*'''Unfavorable histology'''<br>
*'''[[DNA]] index=1'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree | | | | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{familytree | | | | | | C01 |-|+|-| B01 | | | | |C01=<div style="width: 20em; padding:0em;text-align:center">'''Intermediate risk neuroblastoma patients (group 4)'''</div>|B01=<div style="width: 20em; padding:0em;text-align:left">
*'''INSS stage 3'''<br>
*'''365-547 days of [[age]]'''<br>
*'''Unfavorable [[histology]]'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree | | | | | | | | | |!| | | | | | | | | | | | | | | | | }}
{{familytree | | | | | | | | | |`|-| C02 | | | | | | |C02=<div style="width: 20em; padding:0em;text-align:left">
*'''INNS stage 4'''<br>
*'''365-547 days of [[age]]'''<br>
*'''Favorable [[histology]]'''<br>
*'''[[DNA]] index>1'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree/end}}
<br>
{{familytree/start |summary=PE diagnosis Algorithm.}}
{{familytree | | | | | | | | |,|-| A01 | | | | |A01=
<div style="width: 20em; padding:0em;text-align:left">
*'''INNS stage 2A/2B, Stage 3 , Stage 4 or Stage 4S'''<br>
*'''Irrespective of [[age]]'''<br>
*'''Irrespective of grade'''<br>
*'''''MYCN'' amplified'''</div>}}
{{familytree | | | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{familytree | | | | | | | | |)|-| B01 | | | | ||B01=<div style="width: 20em; padding:0em;text-align:left">
*'''INSS stage 3'''<br>
*'''>547 days of [[age]]'''<br>
*'''Unfavorable [[histology]]'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree | | | | | C01 |-|(| | | | | |C01=<div style="width: 20em; padding:1em;text-align:center">'''High risk neuroblastoma [[patients]] (group 5)'''</div> | | | | | | | | | | | | }}
{{familytree | | | | | | | | |)|-| C02 | | | | | | |C02=<div style="width: 20em; padding:0em;text-align:left">
*'''INNS stage 4'''<br>
*'''365-547 days of [[age]]'''<br>
*'''Unfavorable [[histology]]'''<br>
*'''[[DNA]] index=1'''<br>
*'''''MYCN'' not amplified'''</div>}}
{{familytree | | | | | | | | |!| | | | | | | | | | | | | | | | | }}
{{familytree | | | | | | | | |`|-| C02 | | | | | | |C02=<div style="width: 20em; padding:0em;text-align:left">
*'''INNS stage 4'''<br>
*'''>547 days of [[age]]'''<br>
*'''Irrespective grade'''<br>
*'''Irrespective ''MYCN'' [[amplification]] state'''</div>}}
{{familytree/end}}


==References==
==References==
{{reflist|2}}


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Latest revision as of 15:15, 4 March 2019

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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.[1][2][3][4][5]
Stage Description
Stage 1
Stage 2A
Stage 2B
Stage 3
or
  • Unresectable unilateral tumor infiltrating across the midline with positive or negative regional lymph node involvement
or
Stage 4
Stage 4S

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.[2][6][7][8]

Stage Description
Stage L1
Stage L2
Stage M
Stage MS

Risk Stratification

Children's Oncology Group Risk Stratification


 
 
 
 
 
 
 
 
 
 
 
 
  • 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

  1. ↑ 1.0 1.1 Davidoff AM (2012). "Neuroblastoma". Semin Pediatr Surg. 21 (1): 2–14. doi:10.1053/j.sempedsurg.2011.10.009. PMC 3261589. PMID 22248965.
  2. ↑ 2.0 2.1 2.2 Colon NC, Chung DH (2011). "Neuroblastoma". Adv Pediatr. 58 (1): 297–311. doi:10.1016/j.yapd.2011.03.011. PMC 3668791. PMID 21736987.
  3. ↑ H. Shimada, S. Umehara, Y. Monobe, Y. Hachitanda, A. Nakagawa, S. Goto, R. B. Gerbing, D. O. Stram, J. N. Lukens & K. K. Matthay (2001). "International neuroblastoma pathology classification for prognostic evaluation of patients with peripheral neuroblastic tumors: a report from the Children's Cancer Group". Cancer. 92 (9): 2451–2461. PMID 11745303. Unknown parameter |month= ignored (help)
  4. ↑ D. de Sa (2000). "Neuroblastoma classification". Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society. 3 (5): 506–507. PMID 10890940. Unknown parameter |month= ignored (help)
  5. ↑ S. Goto, S. Umehara, R. B. Gerbing, D. O. Stram, G. M. Brodeur, R. C. Seeger, J. N. Lukens, K. K. Matthay & H. Shimada (2001). "Histopathology (International Neuroblastoma Pathology Classification) and MYCN status in patients with peripheral neuroblastic tumors: a report from the Children's Cancer Group". Cancer. 92 (10): 2699–2708. PMID 11745206. Unknown parameter |month= ignored (help)
  6. ↑ Susan L. Cohn, Andrew D. J. Pearson, Wendy B. London, Tom Monclair, Peter F. Ambros, Garrett M. Brodeur, Andreas Faldum, Barbara Hero, Tomoko Iehara, David Machin, Veronique Mosseri, Thorsten Simon, Alberto Garaventa, Victoria Castel & Katherine K. Matthay (2009). "The International Neuroblastoma Risk Group (INRG) classification system: an INRG Task Force report". Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 27 (2): 289–297. doi:10.1200/JCO.2008.16.6785. PMID 19047291. Unknown parameter |month= ignored (help)
  7. ↑ Tom Monclair, Garrett M. Brodeur, Peter F. Ambros, Herve J. Brisse, Giovanni Cecchetto, Keith Holmes, Michio Kaneko, Wendy B. London, Katherine K. Matthay, Jed G. Nuchtern, Dietrich von Schweinitz, Thorsten Simon, Susan L. Cohn & Andrew D. J. Pearson (2009). "The International Neuroblastoma Risk Group (INRG) staging system: an INRG Task Force report". Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 27 (2): 298–303. doi:10.1200/JCO.2008.16.6876. PMID 19047290. Unknown parameter |month= ignored (help)
  8. ↑ Sidharth Mahapatra & Kishore B.. Challagundla (2018). "Cancer, Neuroblastoma". PMID 28846355. Unknown parameter |month= ignored (help)
  9. ↑ Neuroblastoma treatment–for health professionals. National Cancer Institute (2015) http://www.cancer.gov/types/neuroblastoma/hp/neuroblastoma-treatment-pdq#section/_1 Accessed on October, 8 2015
  10. ↑ Nadja C. Colon & Dai H. Chung (2011). "Neuroblastoma". Advances in pediatrics. 58 (1): 297–311. doi:10.1016/j.yapd.2011.03.011. PMID 21736987.


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