Esthesioneuroblastoma

Discussion:

Esthesioneuroblastoma is a tumor arising from the basal layer of the olfactory epithelium in the superior recess of the nasal cavity.

Usually, this tumor begins as a mass in the superior olfactory recess and initially involves the anterior and middle ethmoid air-cells on one side. As it grows, it tends to destroy surrounding bone, and can extend in any direction. 

Some relevant aspects of this tumor:

  • peaks in young adult patients (~2nd decade) and another peak in the 5th to 6th decades
  • nasal stuffiness and rhinorrhea or epistaxis are also referred as primary symptoms 
  • arises from the basal layer of the olfactory epithelium in the superior recess of the nasal cavity
  • slow growing tumor 
  • tends to destroy surrounding bone, and can extend in any direction
  • on imaging they usually present as a soft tissue mass in the superior olfactory recess involving the anterior and middle ethmoid air cells on one side and extending through the cribriform plate into the anterior cranial fossa. 
  • contrast enhancement is often marked in both CT and MRI studies. 
  • treatment usually involves combined chemotherapy and/or radiotherapy with surgical excision.
  • prognosis is significantly affected by presence of distant metastases
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