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Difference between revisions of "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]]. | |||
''Neuroblastoma'' is dealt with in the ''[[neuroblastoma]]'' article. | |||
==General== | |||
Epidemiology:<ref name=pmid20596981/> | |||
*Prognosis: poor. | |||
*Wide age range with bimodal distribution - teens and 60s. | |||
*No sex predilection. | |||
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> | |||
*Nasal obstruction ~ 70%. | |||
*Epistaxis ~ 50%. | |||
*Anosmia. | |||
*Headache. | |||
==Gross== | |||
*Arises from olfactory mucosa - upper nasal cavity.<ref name=Ref_WMSP41>{{Ref WMSP|41}}</ref> | |||
==Microscopic== | |||
Features:<ref name=pmid20596981/> | |||
*[[Small round cell tumour|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).<ref name=Ref_WMSP41>{{Ref WMSP|41}}</ref> | |||
DDx: | |||
*[[Lymphoma]]. | |||
*[[Small cell carcinoma]]. | |||
*Other [[small round cell tumours]]. | |||
*Basaloid squamous carcinoma.{{fact}} | |||
Images: | |||
*[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> | |||
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2811627/figure/Fig6/ Olfactory neuroblastoma (nih.gov)].<ref name=pmid20596981/> | |||
==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.<ref name=Ref_WMSP41>{{Ref WMSP|41}}</ref> | |||
==See also== | |||
*[[Small round cell tumours]]. | |||
*[[Head and neck pathology]]. | |||
==References== | |||
{{Reflist|1}} | |||
[[Category:Head and neck pathology]] | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||