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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== | |||
*S-100: | |||
**Small round cells -ve. | |||
**Sustentacular cells +ve. | |||
***Neuronal ONB subtype is enriched for the presence of S100 sustentacular cells. | |||
*[[Neuroendocrine markers]] +ve (CD56, synaptophysin). | |||
**lower expression of neuroendocrine markers in IDH2 mutant tumors. | |||
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> | |||
**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]] | |||