Difference between revisions of "Olfactory neuroblastoma"

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(Molecular Data added)
(→‎IHC: differences based on mutation status)
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**Small round cells -ve.
**Small round cells -ve.
*[[Neuroendocrine markers]] +ve (CD56, synaptophysin).
*[[Neuroendocrine markers]] +ve (CD56, synaptophysin).
**lower expression of neuroendocrine markers in IDH2 mutant tumors.
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*AE1/AE3 usu. -ve (r/o carcinoma).
*AE1/AE3 usu. -ve (r/o carcinoma).
*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.

Revision as of 02:42, 22 October 2019

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

Neuroblastoma is dealt with in the neuroblastoma article.



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

Clinical presentation:[1]

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


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



  • 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]




  • S-100:
    • Sustentacular cells +ve.
    • Small round cells -ve.
  • Neuroendocrine markers +ve (CD56, synaptophysin).
    • lower expression of neuroendocrine markers in IDH2 mutant tumors.


  • CD45 -ve (r/o lymphoma).
  • AE1/AE3 usu. -ve (r/o carcinoma).
  • CAM5.2 usu. -ve -- up to 35% +ve.[2]
    • Higher expression of cytokeratins in IDH2 mutant tumors.


  • Two major subgroups.[4][5]
    • Classical.
    • G-CIMP +ve with frequent IDH2 R172 mutations.

See also


  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.
  4. Capper, D.; Engel, NW.; Stichel, D.; Lechner, M.; Glöss, S.; Schmid, S.; Koelsche, C.; Schrimpf, D. et al. (08 2018). "DNA methylation-based reclassification of olfactory neuroblastoma.". Acta Neuropathol 136 (2): 255-271. doi:10.1007/s00401-018-1854-7. PMID 29730775.
  5. Classe, M.; Yao, H.; Mouawad, R.; Creighton, CJ.; Burgess, A.; Allanic, F.; Wassef, M.; Leroy, X. et al. (10 2018). "Integrated Multi-omic Analysis of Esthesioneuroblastomas Identifies Two Subgroups Linked to Cell Ontogeny.". Cell Rep 25 (3): 811-821.e5. doi:10.1016/j.celrep.2018.09.047. PMID 30332658.