- Also called" Esthesioneuroblastoma", Neuroepithelial origin
- Age: Bimodal, 20－30 years and 60－70 years
- Sex: Male predilection
- Incidence: 2-3% of primary tumors that involve nasal epithelium
- Locations: Roof of the nasal vault, upper third of nasal septum, superior turbinate, superior middle turbinate
Clinical: Slow growing tumors, nasal obstruction, anosmia, epistaxis, rhinorrhea, orbital invlovement or brain involvement
Neuroimaging: Homogeneous mass with uniform vascular enhancement
Histopathology: Neuroepithelial cells arranged in the classic pseudorosette pattern with fibrillar intracellular background, marked microvascularity. The cells are round or fusiform with few cytoplasms. Homer-Wright or Flexner like substances scatter within the tumor.
Immunohistochemistry: Synaptophysin +ve, MAP-2 +ve, Chromogranin A +ve, NSE +ve, CK +ve is less common, CEA -ve, EMA -ve, GFAP -ve, S100 +ve in isolated cells, CD99 +ve
DDX: Differential diagnosis include "Round blue cell tumors", lymphoma,Embryonal rhabdomyosarcoma, PNET/Ewing's sarcoma,Nasopharyngeal carcinoma,Sinonasal undifferentiated carcinoma, malignant melanoma
References: Greenfield's Neuropathology,Sixth Edition
1: Chin Med J 2007 Feb 5;120(3):224-7. Management of intracranial invasive olfactory neuroblastoma.