Difference between revisions of "Neuroblastoma"

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#redirect [[Adrenal gland#Neuroblastoma]]
'''Neuroblastoma''' is a [[malignant]] tumour of the [[adrenal gland]].
 
''[[Olfactory neuroblastoma]]'' is dealt with separately.
 
==General==
Epidemiology:
*Usually paediatric population.
 
Laboratory findings:
*Increased urine homovanillic acid.
 
Imaging:
*mIBG uptake (>90% neuroblastomas)
 
 
Predictors of a poor prognosis:<ref name=Ref_PCPBoD8_254>{{Ref PCPBoD8|254}}</ref>
*High mitotic-karyorrhectic index.
*Lack of schwannian stroma.
*>18 months.
*Near ploidy.
*N-MYC amplification.
*1p deletion /imbalance
*Lymph node spread.
*Distant spread.
 
Classification:
*In a grouping known as ''neuroblastic tumours'' which includes:<ref name=pmid10421272>{{cite journal |author=Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B |title=Terminology and morphologic criteria of neuroblastic tumors: recommendations by the International Neuroblastoma Pathology Committee |journal=Cancer |volume=86 |issue=2 |pages=349–63 |year=1999 |month=July |pmid=10421272 |doi= |url=}}</ref>
**[[Ganglioneuroma]] (benign).
**[[Ganglioneuroblastoma]] (intermediate).
**Neuroblastoma (aggressive).
 
==Gross==
*Typically an abdominal mass.
**~40% arise in the [[adrenal gland]].<ref name=Ref_PCPBoD8_253>{{Ref PCPBoD8|253}}</ref>
 
==Microscopic==
Features:<ref name=pmid18635637>{{cite journal |author=Chung EM, Murphey MD, Specht CS, Cube R, Smirniotopoulos JG |title=From the Archives of the AFIP. Pediatric orbit tumors and tumorlike lesions: osseous lesions of the orbit |journal=Radiographics |volume=28 |issue=4 |pages=1193–214 |year=2008 |pmid=18635637 |doi=10.1148/rg.284085013 |url=}}</ref>
*[[small round cell tumour|Small round blue cell]]s separated by thin (pink) fibrous septa.
*Homer-Wright rosettes.
**Rosette with a small (~100 micrometers - diameter) meshwork of fibers (neuropil) at the centre.<ref name=pmid16551982>{{cite journal |author=Wippold FJ, Perry A |title=Neuropathology for the neuroradiologist: rosettes and pseudorosettes |journal=AJNR Am J Neuroradiol |volume=27 |issue=3 |pages=488–92 |year=2006 |month=March |pmid=16551982 |doi= |url=}}</ref>
*Neuropil-like stroma = paucicellular stroma with a cotton candy-like appearance; see comparison below.
**>50% neuropil-like stroma -- otherwise it's a [[ganglioneurona]] or ganglioblastoma.
 
Notes:
*The fibrous septa are especially useful for differentiation from lymphoma.
 
DDx:
*[[Small round cell tumours]].
**[[Wilms tumour]].
**Lymphoma.
**[[Hepatoblastoma]].
 
===Images===
<gallery>
Image:Adrenal Neuroblastoma MP CTR.jpg|Neuroblastoma - medium power]]
Image:Adrenal Neuroblastoma 2 MP CTR.jpg|Neuroblastoma - medium power (SKB)
Image:Adrenal Neuroblastoma M2P PA.JPG|Neuroblastoma  - medium power]]
Image:Adrenal Neuroblastoma VascularInvasion MP CTR.jpg|thumb|Neuroblastoma  - vascular invasion - medium power]]
Image:Adrenal Neuroblastoma MP3 PA.JPG|Neuroblastoma  - medium power]]
Image:Adrenal Neuroblastoma MP PA.JPG|Neuroblastoma  - medium power]]
Image:Adrenal Neuroblastoma 2 HP CTR.jpg|Neuroblastoma - high power (SKB)
Image:Adrenal Neuroblastoma 3 HP CTR.jpg|Neuroblastoma  - high power - blue cells arrayed around a core of fluffy pink neuropil (SKB)
Image:Adrenal Neuroblastoma HP2 CTR.jpg|Neuroblastoma -  high power]]
Image:Adrenal Neuroblastoma HP CTR.jpg|Neuroblastoma  -  high power]]
</gallery>
*[http://radiographics.rsna.org/content/28/4/1193/F42.expansion Neuroblastoma (radiographics.rsna.org)].<ref>URL: [http://radiographics.rsna.org/content/28/4/1193.full http://radiographics.rsna.org/content/28/4/1193.full]. Accessed on: 12 January 2011.</ref>
*[http://commons.wikimedia.org/wiki/File:Neuroblastoma_rosettes.jpg Neuroblastoma (WC)].
*[http://farm3.static.flickr.com/2259/2274260465_afbea05f9b.jpg Neuroblastoma (flickr.com)].
 
===Schwannian vs. neuropil===
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
| Feature
| Schwannian
| Neuropil
|-
| Cellularity
| high ~ spacing of cells < 30 µm
| low ~ spacing of cells > 100 µm
|-
| Fibrillary
| yes, long fine strands
| no
|-
| Associations
| ganglion cells
| neuroblasts
|-
| Cytoplasmic vacuolation
| yes
| ?
|-
|}
 
===Classification/grading===
Commonly grouped by the ''Shimada classification'', which depends on the presence a number of things including:
*Mitoses/karyorrhectic cells.
*Molecular abnormalities.
 
International Neuroblastoma Risk Group Staging System (INRGSS)
* L1: Locoregional tumor without Image-Definging Risk factors (IDRFs)
* L2: Locoregional tumor with one or more IDRFs
* M: Distant metastatic disease (except Ms)
* Ms: INRG Stage L1 or L2 tumor with metastatic disease confined to skin and/or liver and/or bone marrow and age below 18 months.
 
==IHC==
*PGP 9.5 +ve.<ref>{{Cite journal  | last1 = Ootsuka | first1 = S. | last2 = Asami | first2 = S. | last3 = Sasaki | first3 = T. | last4 = Yoshida | first4 = Y. | last5 = Nemoto | first5 = N. | last6 = Shichino | first6 = H. | last7 = Chin | first7 = M. | last8 = Mugishima | first8 = H. | last9 = Suzuki | first9 = T. | title = Useful markers for detecting minimal residual disease in cases of neuroblastoma. | journal = Biol Pharm Bull | volume = 31 | issue = 6 | pages = 1071-4 | month = Jun | year = 2008 | doi =  | PMID = 18520032 }}</ref>
**PGP = protein gene product.
*NB-84 +ve.<ref name=pmid9500774>{{Cite journal  | last1 = Miettinen | first1 = M. | last2 = Chatten | first2 = J. | last3 = Paetau | first3 = A. | last4 = Stevenson | first4 = A. | title = Monoclonal antibody NB84 in the differential diagnosis of neuroblastoma and other small round cell tumors. | journal = Am J Surg Pathol | volume = 22 | issue = 3 | pages = 327-32 | month = Mar | year = 1998 | doi =  | PMID = 9500774 }}</ref>
**More sensitive that synaptophysin.
*Synaptophysin +ve.
*CD99 -ve.
 
==EM==
Distinctive EM appearance:<ref name=pmid1196755>{{Cite journal  | last1 = Mackay | first1 = B. | last2 = Masse | first2 = SR. | last3 = King | first3 = OY. | last4 = Butler | first4 = J. | title = Diagnosis of neuroblastoma by electron microscopy of bone marrow aspirates. | journal = Pediatrics | volume = 56 | issue = 6 | pages = 1045-9 | month = Dec | year = 1975 | doi =  | PMID = 1196755 }}</ref>
*Dendritic processes with longitudinally oriented microtubules.
*Membrane bound electron-dense granules (contain catecholamines).
*Desmosomes
**Not seen in [[EWS]], [[RMS]], lymphomas.
*Membrane densities.
 
Pertinent negative:<ref name=pmid1196755/>
*No glycogen.
**Seen in [[EWS]].
 
==See also==
*[[Adrenal gland]].
 
==References==
{{Reflist|1}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Adrenal gland]]

Latest revision as of 09:40, 7 March 2019

Neuroblastoma is a malignant tumour of the adrenal gland.

Olfactory neuroblastoma is dealt with separately.

General

Epidemiology:

  • Usually paediatric population.

Laboratory findings:

  • Increased urine homovanillic acid.

Imaging:

  • mIBG uptake (>90% neuroblastomas)


Predictors of a poor prognosis:[1]

  • High mitotic-karyorrhectic index.
  • Lack of schwannian stroma.
  • >18 months.
  • Near ploidy.
  • N-MYC amplification.
  • 1p deletion /imbalance
  • Lymph node spread.
  • Distant spread.

Classification:

Gross

Microscopic

Features:[4]

  • Small round blue cells separated by thin (pink) fibrous septa.
  • Homer-Wright rosettes.
    • Rosette with a small (~100 micrometers - diameter) meshwork of fibers (neuropil) at the centre.[5]
  • Neuropil-like stroma = paucicellular stroma with a cotton candy-like appearance; see comparison below.
    • >50% neuropil-like stroma -- otherwise it's a ganglioneurona or ganglioblastoma.

Notes:

  • The fibrous septa are especially useful for differentiation from lymphoma.

DDx:

Images

  • Neuroblastoma - medium power]]

  • Neuroblastoma - medium power (SKB)

  • Neuroblastoma - medium power]]

  • Neuroblastoma - vascular invasion - medium power]]

  • Neuroblastoma - medium power]]

  • Neuroblastoma - medium power]]

  • Neuroblastoma - high power (SKB)

  • Neuroblastoma - high power - blue cells arrayed around a core of fluffy pink neuropil (SKB)

  • Neuroblastoma - high power]]

  • Neuroblastoma - high power]]

Schwannian vs. neuropil

Feature Schwannian Neuropil
Cellularity high ~ spacing of cells < 30 µm low ~ spacing of cells > 100 µm
Fibrillary yes, long fine strands no
Associations ganglion cells neuroblasts
Cytoplasmic vacuolation yes ?

Classification/grading

Commonly grouped by the Shimada classification, which depends on the presence a number of things including:

  • Mitoses/karyorrhectic cells.
  • Molecular abnormalities.

International Neuroblastoma Risk Group Staging System (INRGSS)

  • L1: Locoregional tumor without Image-Definging Risk factors (IDRFs)
  • L2: Locoregional tumor with one or more IDRFs
  • M: Distant metastatic disease (except Ms)
  • Ms: INRG Stage L1 or L2 tumor with metastatic disease confined to skin and/or liver and/or bone marrow and age below 18 months.

IHC

  • PGP 9.5 +ve.[7]
    • PGP = protein gene product.
  • NB-84 +ve.[8]
    • More sensitive that synaptophysin.
  • Synaptophysin +ve.
  • CD99 -ve.

EM

Distinctive EM appearance:[9]

  • Dendritic processes with longitudinally oriented microtubules.
  • Membrane bound electron-dense granules (contain catecholamines).
  • Desmosomes
    • Not seen in EWS, RMS, lymphomas.
  • Membrane densities.

Pertinent negative:[9]

  • No glycogen.

See also

References

  1. ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 254. ISBN 978-1416054542.
  2. ↑ Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B (July 1999). "Terminology and morphologic criteria of neuroblastic tumors: recommendations by the International Neuroblastoma Pathology Committee". Cancer 86 (2): 349–63. PMID 10421272.
  3. ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 253. ISBN 978-1416054542.
  4. ↑ Chung EM, Murphey MD, Specht CS, Cube R, Smirniotopoulos JG (2008). "From the Archives of the AFIP. Pediatric orbit tumors and tumorlike lesions: osseous lesions of the orbit". Radiographics 28 (4): 1193–214. doi:10.1148/rg.284085013. PMID 18635637.
  5. ↑ Wippold FJ, Perry A (March 2006). "Neuropathology for the neuroradiologist: rosettes and pseudorosettes". AJNR Am J Neuroradiol 27 (3): 488–92. PMID 16551982.
  6. ↑ URL: http://radiographics.rsna.org/content/28/4/1193.full. Accessed on: 12 January 2011.
  7. ↑ Ootsuka, S.; Asami, S.; Sasaki, T.; Yoshida, Y.; Nemoto, N.; Shichino, H.; Chin, M.; Mugishima, H. et al. (Jun 2008). "Useful markers for detecting minimal residual disease in cases of neuroblastoma.". Biol Pharm Bull 31 (6): 1071-4. PMID 18520032.
  8. ↑ Miettinen, M.; Chatten, J.; Paetau, A.; Stevenson, A. (Mar 1998). "Monoclonal antibody NB84 in the differential diagnosis of neuroblastoma and other small round cell tumors.". Am J Surg Pathol 22 (3): 327-32. PMID 9500774.
  9. ↑ 9.0 9.1 Mackay, B.; Masse, SR.; King, OY.; Butler, J. (Dec 1975). "Diagnosis of neuroblastoma by electron microscopy of bone marrow aspirates.". Pediatrics 56 (6): 1045-9. PMID 1196755.
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