View source for Olfactory neuroblastoma

{{ Infobox diagnosis
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| Image      = Esthesioneuroblastoma HE.jpg 
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| Caption    = Olfactory neuroblastoma
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| Micro      = [[Small round cell tumour|small round (blue) cell tumour]] (stippled chromatin, high NC ratio),
+/-Flexner-Wintersteiner [[rosette]] (rosette with empty centre), +/-Fibrillary, eosinophilic material (neuropil-like)
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| LMDDx      = other small round blue cell tumours (e.g. [[small cell carcinoma of the lung]]), large cell lymphoma (e.g. [[DLBCL]]), basaloid squamous cell carcinoma
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| Prevalence = rare
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| Prognosis  = poor
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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/>

<gallery>
File:Esthesioneuroblastoma HE.jpg | Olfactory neuroblastoma (H&E).
File:Olfactory neuroblastoma.jpg | Olfactory neuroblastoma (AFIP).
File:Esthesioneuroblastoma.jpg | Sustenticular cells in ONB (AFIP).
</gallery>

==IHC==
*[[Neuroendocrine markers]]:<ref name=pmid36452592>{{cite journal |vauthors=Rao KR, Upadhya IB |title=A Review on Esthesioneuroblastoma |journal=Indian J Otolaryngol Head Neck Surg |volume=74 |issue=Suppl 2 |pages=1584–1590 |date=October 2022 |pmid=36452592 |pmc=9702120 |doi=10.1007/s12070-021-02726-2 |url=}}</ref>
**CD56 +ve.
**Synaptophysin +ve. 
**Chromogranin A +ve.
**NSE +ve.
***Lower expression of neuroendocrine markers in IDH2 mutant tumors.{{fact}}
*S-100:
**Small round cells -ve.
**Sustentacular cells +ve.
***Neuronal ONB subtype is enriched for the presence of S100 sustentacular cells.

Others:
*CD45 -ve (r/o [[lymphoma]]).
*AE1/AE3 usually -ve (~1/3 are +ve<ref name=pmid36452592/>).
*CAM5.2 usu. -ve -- up to 35% +ve.<ref name=Ref_WMSP41>{{Ref WMSP|41}}</ref>
**Higher expression of cytokeratins in IDH2 mutant tumors.

==Molecular==
* Two major subgroups.<ref>{{Cite journal  | last1 = Capper | first1 = D. | last2 = Engel | first2 = NW. | last3 = Stichel | first3 = D. | last4 = Lechner | first4 = M. | last5 = Glöss | first5 = S. | last6 = Schmid | first6 = S. | last7 = Koelsche | first7 = C. | last8 = Schrimpf | first8 = D. | last9 = Niesen | first9 = J. | title = DNA methylation-based reclassification of olfactory neuroblastoma. | journal = Acta Neuropathol | volume = 136 | issue = 2 | pages = 255-271 | month = 08 | year = 2018 | doi = 10.1007/s00401-018-1854-7 | PMID = 29730775 }}</ref><ref>{{Cite journal  | last1 = Classe | first1 = M. | last2 = Yao | first2 = H. | last3 = Mouawad | first3 = R. | last4 = Creighton | first4 = CJ. | last5 = Burgess | first5 = A. | last6 = Allanic | first6 = F. | last7 = Wassef | first7 = M. | last8 = Leroy | first8 = X. | last9 = Verillaud | first9 = B. | title = Integrated Multi-omic Analysis of Esthesioneuroblastomas Identifies Two Subgroups Linked to Cell Ontogeny. | journal = Cell Rep | volume = 25 | issue = 3 | pages = 811-821.e5 | month = 10 | year = 2018 | doi = 10.1016/j.celrep.2018.09.047 | PMID = 30332658 }}</ref>
**Classical ("neural").
**Basal: G-CIMP+ve with frequent IDH2 R172 mutations.

==See also==
*[[Small round cell tumours]].
*[[Head and neck pathology]].

==References==
{{Reflist|1}}


[[Category:Head and neck pathology]]
[[Categorgy:Neuropathology]]
[[Category:Diagnosis]]