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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 mutations.
* 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.
* 1-2% of neuroblastoma cases may demonstrate a familial predilection.
* Genes involved in the pathogenesis of neuroblastoma include:
* Genes involved in the pathogenesis of neuroblastoma include:
:* ''ALK'' gene on chromosome 2
:* ''NBPF10'' gene [[chromosome 1]]
:* ''KIF1B'' [[gene]] on chromosome 1
:* ''ALK'' gene on [[chromosome 2]]
:* ''LMO1'' gene on [[chromosome 11]]
:* ''PHOX2A'' gene on chromosome 11
:* ''PHOX2A'' gene on chromosome 11
:* ''KIF1B'' gene on chromosome 1
* ''MYCN'' [[oncogene]] amplification is a common finding among neuroblastoma patients.
:* ''LMO1'' gene on chromosome 11
 
:* ''NBPF10'' gene chromosome 1
==Associated Conditions==
* MYCN oncogene amplification is a common finding among neuroblastoma patients.
* 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'''
 
! 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 [[neuroblast]]s 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 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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