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==Pathogenesis==
==Pathogenesis==
* Neuroblastoma arises from neural crest cells, which are normally involved in the development of the sympathetic nervous system and adrenal glands.
* Neuroblastoma arises from [[neural crest]] cells, which are normally involved in the development of the [[sympathetic nervous system]] and [[adrenal gland]]s.<ref name="wiki>Neuroblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 5 2015</ref><ref name="radio">Neuroblastoma. Radiopaedia(2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 5 2015</ref>
* Neuroblastoma is frequently observed along the sympathetic nervous system structures. Specific sites may include:
* Neuroblastoma is frequently observed along the sympathetic nervous system structures. Specific sites may include:
:* Adrenal glands (35% of the cases)
:* Adrenal glands (35% of the cases)
:* Retroperitoneal organs (30% of the cases):
:* [[Retroperitoneal]] organs (30% of the cases):
::* Organ of Zuckerkandl
::* [[Organ of Zuckerkandl]]
::* Coeliac axis
::* Coeliac axis
::* Paravertebral sympathetic chain
::* Paravertebral [[sympathetic chain]]
:* Posterior mediastinum (20% of the cases)  
:* Posterior [[mediastinum]] (20% of the cases)  
:* Nerve tissues in the neck (1-5% of the cases)  
:* [[Nerve]] tissues in the neck (1-5% of the cases)  
:* Nerve tissues in the pelvis (2-3% of the cases)
:* Nerve tissues in the [[pelvis]] (2-3% of the cases)
* Neuroblastoma cells can secrete catecholamines such as:
* Neuroblastoma tumor cells secrete [[catecholamine]]s such as:
:* Vanillylmandelic acid (VMA)
:* [[Vanillylmandelic acid]] (VMA)
:* Homovanillic acid (HVA)
:* [[Homovanillic acid]] (HVA)
* Neuroblastoma may demonstrate spontaneous regression from an undifferentiated state to a completely benign cellular state.
* Neuroblastoma may demonstrate spontaneous regression from an undifferentiated state to a completely [[benign]] cellular state.


==Genetics==
==Genetics==
* Development of neuroblasotma is the result of multiple [[genetic mutation]]s.<ref name="radio">Neuroblastoma. Radiopaedia(2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 5 2015</ref><ref name="wiki>Neuroblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 5 2015</ref>
* The vast majority of neuroblastoma cases are sporadic.
* 1-2% of neuroblastoma cases may demonstrate a familial predilection.
* Genes involved in the pathogenesis of neuroblastoma include:
:* ''NBPF10'' gene [[chromosome 1]]
:* ''KIF1B'' [[gene]] on chromosome 1
:* ''ALK'' gene on [[chromosome 2]]
:* ''LMO1'' gene on [[chromosome 11]]
:* ''PHOX2A'' gene on chromosome 11
* ''MYCN'' [[oncogene]] amplification is a common finding among neuroblastoma patients.


==Associated Conditions==
* Neuroblastoma is associated with a number of syndromes that include:<ref name="radio">Neuroblastoma. Radiopaedia(2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 5 2015</ref><ref name="wiki>Neuroblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 5 2015</ref>
:* [[Neurofibromatosis type 1]] (von Recklinghausen disease)
:* [[Beckwith-Wiedemann syndrome]]
:* [[DiGeorge syndrome]]
:* [[Hirschsprung disease]]


==Gross Pathology==
* On gross pathology, a  well defined, bulky, and tan colored mass is a characteristic finding of neuroblastoma.<ref name="radio">Neuroblastoma. Radiopaedia(2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 5 2015</ref><ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref>
* Other associated findings of neuroblastoma on gross pathology may include:
:* Fibrous [[pseudocapsule]]
:* [[Necrosis]]
:* [[Hemorrhage]]
:* [[Calcification]]


==Microscopic Pathology==
* On microscopic histopathological analysis the presence of round blue cells separated by thin [[fibrous]] septa are characteristic findings of neuroblastoma.
* Other findings of neuroblastoma on [[light microscopy]] may include:<ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref>
:* [[Homer-Wright rosette]]s (rosettes with a small  meshwork of fibers at the center)
:* Neuropil-like [[stroma]] ([[paucicellular]] stroma with a cotton candy-like appearance)
* On [[electron microscopy]] neuroblastoma is characterized by:
:* [[Dendritic processes]] with longitudinally oriented [[microtubule]]s
:* Membrane bound electron-dense [[granule]]s that contain [[catecholamine]]s
:* Presence of [[desmosomes]]
:* Absence of [[glycogen]]
* On [[immunohistochemistry]] neuroblastoma is characterized by:
:* Protein gene product (PGP) 9.5 positivity
:* [[Monoclonal antibody]] NB84 positivity
:* [[Synaptophysin]] positivity
:* [[CD99 marker]] negativity
* 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><ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref>
<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:<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>


{|
! style="background: #4479BA; width: 200px; color: #FFFFFF;"|'''Risk Factor'''


[10bout 1–2% of cases run in families and have been linked to specific gene mutations.
! style="background: #4479BA; width: 600px; color: #FFFFFF;"|'''Description'''
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.
|-
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]
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | '''Undifferentiated neruoblastoma'''|| style="padding: 5px 5px; background: #F5F5F5;" | Completely formed by [[neuroblast]]s with no maturity of ganglion cells
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="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 cell]]s
 
|-
 
| 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
|}
 
==Gallery==
* Illustrated below is a series of microscopic images demonstrating neuroblastoma:
<gallery>
Image:
120px-Adrenal Neuroblastoma 2 HP CTR.jpg|Adrenal neuroblastoma<ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref>
Image:
120px-Adrenal Neuroblastoma 2 MP CTR.jpg|Adrenal neuroblastoma<ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref>
Image:
120px-Adrenal Neuroblastoma 3 HP CTR.jpg|Adrenal neuroblastoma<ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref>
Image:
120px-Adrenal Neuroblastoma HP CTR.jpg|Adrenal neuroblastoma<ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref>
Image:
120px-Adrenal Neuroblastoma HP2 CTR.jpg|Adrenal neuroblastoma<ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref>
Image:
120px-Adrenal Neuroblastoma M2P PA.JPG|Adrenal neuroblastoma<ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref>
Image:
120px-Adrenal Neuroblastoma MP CTR.jpg|Adrenal neuroblastoma<ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref>
Image:
120px-Adrenal Neuroblastoma MP PA.JPG|Adrenal neuroblastoma<ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref>
Image:
120px-Adrenal Neuroblastoma MP3 PA.JPG|Adrenal neuroblastoma<ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref>
Image:
120px-Adrenal Neuroblastoma VascularInvasion MP CTR.jpg<ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref>
</gallery>

Latest revision as of 02:14, 6 October 2015

Pathogenesis

  • Adrenal glands (35% of the cases)
  • Retroperitoneal organs (30% of the cases):
  • Posterior mediastinum (20% of the cases)
  • Nerve tissues in the neck (1-5% of the cases)
  • Nerve tissues in the pelvis (2-3% of the cases)
  • Neuroblastoma may demonstrate spontaneous regression from an undifferentiated state to a completely benign cellular state.

Genetics

  • Development of neuroblasotma is the result of multiple genetic mutations.[2][1]
  • The vast majority of neuroblastoma cases are sporadic.
  • 1-2% of neuroblastoma cases may demonstrate a familial predilection.
  • Genes involved in the pathogenesis of neuroblastoma include:
  • MYCN oncogene amplification is a common finding among neuroblastoma patients.

Associated Conditions

  • Neuroblastoma is associated with a number of syndromes that include:[2][1]

Gross Pathology

  • On gross pathology, a well defined, bulky, and tan colored mass is a characteristic finding of neuroblastoma.[2][3]
  • Other associated findings of neuroblastoma on gross pathology may include:

Microscopic Pathology

  • On microscopic histopathological analysis the presence of round blue cells separated by thin fibrous septa are characteristic findings of neuroblastoma.
  • Other findings of neuroblastoma on light microscopy may include:[3]
  • 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:[4][5][3]


 
 
 
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:[4]
  • Undifferentiated neruoblastoma
  • Poorly differentiated neuroblastoma
  • Differentiating neuroblastoma
  • The table below summarizes the differnces between the three histological subtypes of neurublastoma:[4]
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

Gallery

  • Illustrated below is a series of microscopic images demonstrating neuroblastoma:
  • Adrenal neuroblastoma[3]

    Adrenal neuroblastoma[3]

  • Adrenal neuroblastoma[3]

    Adrenal neuroblastoma[3]

  • Adrenal neuroblastoma[3]

    Adrenal neuroblastoma[3]

  • Adrenal neuroblastoma[3]

    Adrenal neuroblastoma[3]

  • Adrenal neuroblastoma[3]

    Adrenal neuroblastoma[3]

  • Adrenal neuroblastoma[3]

    Adrenal neuroblastoma[3]

  • Adrenal neuroblastoma[3]

    Adrenal neuroblastoma[3]

  • Adrenal neuroblastoma[3]

    Adrenal neuroblastoma[3]

  • Adrenal neuroblastoma[3]

    Adrenal neuroblastoma[3]

  1. ↑ 1.0 1.1 1.2 Neuroblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 5 2015
  2. ↑ 2.0 2.1 2.2 2.3 Neuroblastoma. Radiopaedia(2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 5 2015
  3. ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015
  4. ↑ 4.0 4.1 4.2 Neuroblastoma, Ganglioneuroblastoma and Ganglioneuroma. Stanford Medicine Surgical Pathology Criteria(2015) http://surgpathcriteria.stanford.edu/srbc/neuroblastoma-ganglioneuroblastoma-ganglioneuroma/ Accessed on October, 5 2015
  5. ↑ 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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