Olfactory neuroblastoma: Difference between revisions
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Esthesioneuroblastoma HE.jpg | |||
| Width = | |||
| Caption = Olfactory neuroblastoma | |||
| Synonyms = | |||
| 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) | |||
| Subtypes = | |||
| 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 | |||
| Stains = | |||
| IHC = | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Staging = | |||
| Site = | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = rare | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = poor | |||
| Other = | |||
| ClinDDx = | |||
| Tx = | |||
}} | |||
'''Olfactory neuroblastoma''', also known as '''esthesioneuroblastoma''', is a rare aggressive tumour of the [[head and neck pathology|head and neck]]. | '''Olfactory neuroblastoma''', also known as '''esthesioneuroblastoma''', is a rare aggressive tumour of the [[head and neck pathology|head and neck]]. | ||
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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> | *[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/> | *[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== | ==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. | **Sustentacular cells +ve. | ||
** | ***Neuronal ONB subtype is enriched for the presence of S100 sustentacular cells. | ||
Others: | Others: | ||
*CD45 -ve (r/o [[lymphoma]]). | *CD45 -ve (r/o [[lymphoma]]). | ||
*AE1/AE3 | *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> | *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== | ==See also== | ||
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[[Category:Head and neck pathology]] | [[Category:Head and neck pathology]] | ||
[[Category:Neuropathology]] | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||