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It is a neuroendocrine tumor, arising from any neural crest element of the sympathetic nervous system (SNS). It most frequently originates in one of the adrenal glands, but can also develop in nerve tissues in the neck, chest, abdomen, or pelvis.


Neuroblastoma is one of the few human malignancies known to demonstrate spontaneous regression from an undifferentiated state to a completely benign cellular appearance
* Neuroblastic tumors may be grouped according to International Neuroblastoma Pathology Classification (Shimada classification) into two groups: schwannian stroma rich group and a schwannian stroma poor group as illustrated below:<ref name="Stanford">Neuroblastoma, Ganglioneuroblastoma and Ganglioneuroma. Stanford Medicine Surgical Pathology Criteria(2015) http://surgpathcriteria.stanford.edu/srbc/neuroblastoma-ganglioneuroblastoma-ganglioneuroma/ Accessed on October, 5 2015</ref><ref name="pmid11745303">{{cite journal| author=Shimada H, Umehara S, Monobe Y, Hachitanda Y, Nakagawa A, Goto S et al.| title=International neuroblastoma pathology classification for prognostic evaluation of patients with peripheral neuroblastic tumors: a report from the Children's Cancer Group. | journal=Cancer | year= 2001 | volume= 92 | issue= 9 | pages= 2451-61 | pmid=11745303 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11745303  }} </ref>
The most common location for neuroblastoma to originate (i.e., the primary tumor) is on the adrenal glands. This occurs in 40% of localized tumors and in 60% of cases of widespread disease. Neuroblastoma can also develop anywhere along the sympathetic nervous system chain from the neck to the pelvis. Frequencies in different locations include: neck (1%), chest (19%), abdomen (30% non-adrenal), or pelvis (1%). In rare cases, no primary tumor can be discerned.[10bout 1–2% of cases run in families and have been linked to specific gene mutations. Familial neuroblastoma in some cases is caused by rare germline mutations in the anaplastic lymphoma kinase (ALK) gene.[13] Germline mutations in the PHOX2A or KIF1B gene have been implicated in familial neuroblastoma as well. Neuroblastoma is also a feature of neurofibromatosis type 1 and the Beckwith-Wiedemann syndrome.
<br>
{{Family tree/start}}
{{familytree  | | | | Y01 | | | | | | | | | | Y01=<div style="width: 20em; padding:0.5em;">''' Neuroblastic tumors'''</div>}}
{{familytree  | | |,|-|^|-|.| | | | | | | | }}
{{familytree  | | X01 | |  X02 | | | | | | |X01='''Schwannian stroma rich group'''| X02='''Schwannian stroma poor group'''}}
{{familytree|boxstyle= border-top: 0px;| | A01 | |  A03 | | | | | | |A01=<div style="width: 20em; padding:1em;">
'''Undifferentiated neuroblastoma'''<br>'''Poorly differentiated neuroblastoma'''<br>'''Differentiating neuroblastoma'''</div>|A03=<div style="width: 20em; padding:1em;">|A03=<div style="width: 20em; padding:1em;">
'''Nodular ganglioneuroblastoma'''<br>'''Intermixed ganglioneuroblastoma'''<br>'''Maturing ganglioneuroma'''<br>'''Mature ganglioneuroma''' </div>}}
{{Family tree/end}}
<br>
* Based on the degree of the cellular maturity and composition, neuroblastoma may be further classified into three subtypes according to the International Neuroblastoma Pathology Classification which include:<ref name="Stanford">Neuroblastoma, Ganglioneuroblastoma and Ganglioneuroma. Stanford Medicine Surgical Pathology Criteria(2015) http://surgpathcriteria.stanford.edu/srbc/neuroblastoma-ganglioneuroblastoma-ganglioneuroma/ Accessed on October, 5 2015</ref>
:* Undifferentiated neruoblastoma
:* Poorly differentiated neuroblastoma
:* Differentiating neuroblastoma
* The table below summarizes the differnces between the three histological subtypes of neurublastoma:


MYCN oncogene amplification within the tumor is a common finding in neuroblastoma. The degree of amplification shows a bimodal distribution: either 3- to 10-fold, or 100- to 300-fold. The presence of this mutation is highly correlated to advanced stages of disease.[14]
{|
Duplicated segments of the LMO1 gene within neuroblastoma tumor cells have been shown to increase the risk of developing an aggressive form of the cancer.[15]Neuroblastoma has been linked to copy-number variation within the NBPF10 gene, which results in the 1q21.1 deletion syndrome or 1q21.1 duplication syndrome.[16]
! style="background: #4479BA; width: 200px; color: #FFFFFF;"|'''Risk Factor'''
 
! style="background: #4479BA; width: 600px; color: #FFFFFF;"|'''Description'''
 
|-
 
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | '''Undifferentiated neruoblastoma'''|| style="padding: 5px 5px; background: #F5F5F5;" | Completely formed by neuroblasts with no maturity of ganglion cells
 
|-
 
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | '''Poorly differentiated neuroblastoma'''|| style="padding: 5px 5px; background: #F5F5F5;" |Mostly formed by neuroblasts with less the 5% maturing ganglion cells
 
|-
 
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | '''Differentiating neuroblastoma'''|| style="padding: 5px 5px; background: #F5F5F5;" |
Predominantly formed by neuroblasts but with more than 5% mature ganglion cells  
|}

Latest revision as of 01:13, 6 October 2015

  • Neuroblastic tumors may be grouped according to International Neuroblastoma Pathology Classification (Shimada classification) into two groups: schwannian stroma rich group and a schwannian stroma poor group as illustrated below:[1][2]


 
 
 
Neuroblastic tumors
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Schwannian stroma rich group
 
Schwannian stroma poor group
 
 
 
 
 
 
 
Undifferentiated neuroblastoma
Poorly differentiated neuroblastoma
Differentiating neuroblastoma
 
Nodular ganglioneuroblastoma
Intermixed ganglioneuroblastoma
Maturing ganglioneuroma
Mature ganglioneuroma
 
 
 
 
 
 


  • Based on the degree of the cellular maturity and composition, neuroblastoma may be further classified into three subtypes according to the International Neuroblastoma Pathology Classification which include:[1]
  • Undifferentiated neruoblastoma
  • Poorly differentiated neuroblastoma
  • Differentiating neuroblastoma
  • The table below summarizes the differnces between the three histological subtypes of neurublastoma:
Risk Factor Description
Undifferentiated neruoblastoma Completely formed by neuroblasts with no maturity of ganglion cells
Poorly differentiated neuroblastoma Mostly formed by neuroblasts with less the 5% maturing ganglion cells
Differentiating neuroblastoma

Predominantly formed by neuroblasts but with more than 5% mature ganglion cells

  1. ↑ 1.0 1.1 Neuroblastoma, Ganglioneuroblastoma and Ganglioneuroma. Stanford Medicine Surgical Pathology Criteria(2015) http://surgpathcriteria.stanford.edu/srbc/neuroblastoma-ganglioneuroblastoma-ganglioneuroma/ Accessed on October, 5 2015
  2. ↑ Shimada H, Umehara S, Monobe Y, Hachitanda Y, Nakagawa A, Goto S; et al. (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): 2451–61. PMID 11745303.
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