Olfactory neuroblastoma

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

Neuroblastoma is dealt with in the neuroblastoma article.

General

Epidemiology:[1]

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

Clinical presentation:[1]

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

Gross

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

Microscopic

Features:[1]

  • 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]

DDx:

Images:

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.[2]
    • Higher expression of cytokeratins in IDH2 mutant tumors.

Molecular

  • Two major subgroups.[4][5]
    • Classical ("neural").
    • Basal: G-CIMP+ve with frequent IDH2 R172 mutations.

See also

References

  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.
Categorgy:Neuropathology