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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 gland]]s. | * 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) | ||
| Line 16: | Line 16: | ||
==Genetics== | ==Genetics== | ||
* Development of neuroblasotma is the result of multiple [[genetic mutation]]s. | * 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. | * 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. | ||
| Line 28: | Line 28: | ||
==Associated Conditions== | ==Associated Conditions== | ||
* Neuroblastoma is associated with a number of syndromes that include: | * 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) | :* [[Neurofibromatosis type 1]] (von Recklinghausen disease) | ||
:* [[Beckwith-Wiedemann syndrome]] | :* [[Beckwith-Wiedemann syndrome]] | ||
| Line 35: | Line 35: | ||
==Gross Pathology== | ==Gross Pathology== | ||
* On gross pathology, a well defined, bulky, and tan colored mass is a characteristic finding of neuroblastoma. | * 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: | * Other associated findings of neuroblastoma on gross pathology may include: | ||
:* Fibrous [[pseudocapsule]] | :* Fibrous [[pseudocapsule]] | ||
| Line 44: | Line 44: | ||
==Microscopic Pathology== | ==Microscopic Pathology== | ||
* On microscopic histopathological analysis the presence of round blue cells separated by thin [[fibrous]] septa are characteristic findings of neuroblastoma. | * 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: | * 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) | :* [[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) | :* Neuropil-like [[stroma]] ([[paucicellular]] stroma with a cotton candy-like appearance) | ||
| Line 57: | Line 57: | ||
:* [[Synaptophysin]] positivity | :* [[Synaptophysin]] positivity | ||
:* [[CD99 marker]] negativity | :* [[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> | * 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> | <br> | ||
{{Family tree/start}} | {{Family tree/start}} | ||
| Line 94: | Line 94: | ||
==Gallery== | ==Gallery== | ||
* Illustrated below is a series of microscopic images | * Illustrated below is a series of microscopic images demonstrating neuroblastoma: | ||
<gallery> | <gallery> | ||
Image: | Image: | ||
Latest revision as of 02:14, 6 October 2015
Pathogenesis
- Neuroblastoma arises from neural crest cells, which are normally involved in the development of the sympathetic nervous system and adrenal glands.[1][2]
- Neuroblastoma is frequently observed along the sympathetic nervous system structures. Specific sites may include:
- Adrenal glands (35% of the cases)
- Retroperitoneal organs (30% of the cases):
- Organ of Zuckerkandl
- Coeliac axis
- Paravertebral sympathetic chain
- 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 tumor cells secrete catecholamines such as:
- Vanillylmandelic acid (VMA)
- Homovanillic acid (HVA)
- 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:
- 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
- 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.[2][3]
- 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:[3]
- Homer-Wright rosettes (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 microtubules
- Membrane bound electron-dense granules that contain catecholamines
- 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:[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]](/images/1/19/120px-Adrenal_Neuroblastoma_2_HP_CTR.jpg)
Adrenal neuroblastoma[3]
-
![Adrenal neuroblastoma[3]](/images/2/2b/120px-Adrenal_Neuroblastoma_2_MP_CTR.jpg)
Adrenal neuroblastoma[3]
-
![Adrenal neuroblastoma[3]](/images/4/43/120px-Adrenal_Neuroblastoma_3_HP_CTR.jpg)
Adrenal neuroblastoma[3]
-
![Adrenal neuroblastoma[3]](/images/e/e7/120px-Adrenal_Neuroblastoma_HP_CTR.jpg)
Adrenal neuroblastoma[3]
-
![Adrenal neuroblastoma[3]](/images/c/cb/120px-Adrenal_Neuroblastoma_HP2_CTR.jpg)
Adrenal neuroblastoma[3]
-
![Adrenal neuroblastoma[3]](/images/e/eb/120px-Adrenal_Neuroblastoma_M2P_PA.JPG)
Adrenal neuroblastoma[3]
-
![Adrenal neuroblastoma[3]](/images/4/46/120px-Adrenal_Neuroblastoma_MP_CTR.jpg)
Adrenal neuroblastoma[3]
-
![Adrenal neuroblastoma[3]](/images/3/3a/120px-Adrenal_Neuroblastoma_MP_PA.JPG)
Adrenal neuroblastoma[3]
-
![Adrenal neuroblastoma[3]](/images/1/1c/120px-Adrenal_Neuroblastoma_MP3_PA.JPG)
Adrenal neuroblastoma[3]
![Adrenal neuroblastoma[3]](/index.php/File:120px-Adrenal_Neuroblastoma_2_HP_CTR.jpg)
![Adrenal neuroblastoma[3]](/index.php/File:120px-Adrenal_Neuroblastoma_2_MP_CTR.jpg)
![Adrenal neuroblastoma[3]](/index.php/File:120px-Adrenal_Neuroblastoma_3_HP_CTR.jpg)
![Adrenal neuroblastoma[3]](/index.php/File:120px-Adrenal_Neuroblastoma_HP_CTR.jpg)
![Adrenal neuroblastoma[3]](/index.php/File:120px-Adrenal_Neuroblastoma_HP2_CTR.jpg)
![Adrenal neuroblastoma[3]](/index.php/File:120px-Adrenal_Neuroblastoma_M2P_PA.JPG)
![Adrenal neuroblastoma[3]](/index.php/File:120px-Adrenal_Neuroblastoma_MP_CTR.jpg)
![Adrenal neuroblastoma[3]](/index.php/File:120px-Adrenal_Neuroblastoma_MP_PA.JPG)
![Adrenal neuroblastoma[3]](/index.php/File:120px-Adrenal_Neuroblastoma_MP3_PA.JPG)
- ↑ 1.0 1.1 1.2 Neuroblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 5 2015
- ↑ 2.0 2.1 2.2 2.3 Neuroblastoma. Radiopaedia(2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 5 2015
- ↑ 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
- ↑ 4.0 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
- ↑ 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.