Esthesioneuroblastoma pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]
Overview
Pathophysiology
Gross Pathology
Arises from olfactory mucosa - upper nasal cavity
Microscopic Pathology
- 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)
Olfactory neuroblastomas are of neural crest cell origin 1. They are mulilobulated pink-grey tumors. Histology demonstrates variable differentiation, from well formed neural tissue to undifferentiated neuroblasts with pseudorosette formation 2. It has been suggested that olfactory neuroblastoma is actually part of the Ewing sarcoma group of tumors, rather than being related to neuroblastoma.
Associated Conditions
- Associated with Trisomy 8.