Difference between revisions of "Olfactory neuroblastoma"

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#redirect [[Head_and_neck_pathology#Olfactory_neuroblastoma]]
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'''Olfactory neuroblastoma''', also known as '''esthesioneuroblastoma''', is a rare aggressive tumour of the [[head and neck pathology|head and neck]].
  
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''Neuroblastoma'' is dealt with in the ''[[neuroblastoma]]'' article.
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==General==
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Epidemiology:<ref name=pmid20596981/>
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*Prognosis: poor.
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*Wide age range with bimodal distribution - teens and 60s.
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*No sex predilection.
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Clinical presentation:<ref name=pmid20596981>{{Cite journal  | last1 = Thompson | first1 = LD. | title = Olfactory neuroblastoma. | journal = Head Neck Pathol | volume = 3 | issue = 3 | pages = 252-9 | month = Sep | year = 2009 | doi = 10.1007/s12105-009-0125-2 | PMID = 20596981 | PMC = 2811627 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2811627/?tool=pubmed }}</ref>
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*Nasal obstruction ~ 70%.
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*Epistaxis ~ 50%.
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*Anosmia.
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*Headache.
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==Gross==
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*Arises from olfactory mucosa - upper nasal cavity.<ref name=Ref_WMSP41>{{Ref WMSP|41}}</ref>
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==Microscopic==
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Features:<ref name=pmid20596981/>
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*[[Small round cell tumour|Small round (blue) cell tumour]] with:
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**Stippled chromatin.
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**High NC ratio.
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*+/-Flexner-Wintersteiner [[rosette]] - rosette with empty centre (donut hole).
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*+/-Fibrillary, eosinophilic material (neuropil-like).<ref name=Ref_WMSP41>{{Ref WMSP|41}}</ref>
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DDx:
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*[[Lymphoma]].
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*[[Small cell carcinoma]].
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*Other [[small round cell tumours]].
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*Basaloid squamous carcinoma.{{fact}}
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Images:
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*[http://path.upmc.edu/cases/case467/images/fig02.jpg Olfactory neuroblastoma - crappy image (upmc.edu)].<ref>URL: [http://path.upmc.edu/cases/case467.html http://path.upmc.edu/cases/case467.html]. Accessed on: 21 January 2012.</ref>
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*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2811627/figure/Fig6/ Olfactory neuroblastoma (nih.gov)].<ref name=pmid20596981/>
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==IHC==
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*S100:
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**Sustentacular cells +ve.
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**Small round cells -ve.
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*Neuroendocrine markers +ve (CD56, synaptophysin).
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Others:
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*CD45 -ve (r/o [[lymphoma]]).
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*AE1/AE3 usu. -ve (r/o carcinoma).
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*CAM5.2 usu. -ve -- up to 35% +ve.<ref name=Ref_WMSP41>{{Ref WMSP|41}}</ref>
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==See also==
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*[[Small round cell tumours]].
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*[[Head and neck pathology]].
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==References==
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{{Reflist|1}}
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[[Category:Head and neck pathology]]
 
[[Category:Diagnosis]]
 
[[Category:Diagnosis]]

Revision as of 01:14, 27 July 2014

Olfactory neuroblastoma, also known as esthesioneuroblastoma, is a rare aggressive tumour of the head and neck.

Neuroblastoma is dealt with in the neuroblastoma article.

General

Epidemiology:[1]

  • Prognosis: poor.
  • Wide age range with bimodal distribution - teens and 60s.
  • No sex predilection.

Clinical presentation:[1]

  • Nasal obstruction ~ 70%.
  • Epistaxis ~ 50%.
  • Anosmia.
  • Headache.

Gross

  • Arises from olfactory mucosa - upper nasal cavity.[2]

Microscopic

Features:[1]

  • Small round (blue) cell tumour with:
    • Stippled chromatin.
    • High NC ratio.
  • +/-Flexner-Wintersteiner rosette - rosette with empty centre (donut hole).
  • +/-Fibrillary, eosinophilic material (neuropil-like).[2]

DDx:

Images:

IHC

  • S100:
    • Sustentacular cells +ve.
    • Small round cells -ve.
  • Neuroendocrine markers +ve (CD56, synaptophysin).

Others:

  • CD45 -ve (r/o lymphoma).
  • AE1/AE3 usu. -ve (r/o carcinoma).
  • CAM5.2 usu. -ve -- up to 35% +ve.[2]

See also

References

  1. 1.0 1.1 1.2 1.3 Thompson, LD. (Sep 2009). "Olfactory neuroblastoma.". Head Neck Pathol 3 (3): 252-9. doi:10.1007/s12105-009-0125-2. PMC 2811627. PMID 20596981. //www.ncbi.nlm.nih.gov/pmc/articles/PMC2811627/.
  2. 2.0 2.1 2.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 41. ISBN 978-0781765275.
  3. URL: http://path.upmc.edu/cases/case467.html. Accessed on: 21 January 2012.