Olfactory neuroblastoma (esthesioneuroblastoma)

Case contributed by Assoc Prof Frank Gaillard

Presentation

Rhinorrhea and episodes of epistaxis.

Patient Data

Age: 50 years
Gender: Male
CT

Paransal sinuses

CT and MRI through the paranasal sinuses demonstrate a large aggressive mass the epicenter of which appears to be centered in the superior aspect of the nasal cavity.

The mass extends superiorly into the anterior cranial fossa, laterally into the orbit and posteriorly into the sphenoid sinus. The outflow of the sphenoid sinus and left frontal sinus is presumably obstructed as the sinuses are opacified. 

The mass is of soft tissue density, intermediate signal on T1 and T2 weighted images, and demonstrates extensive heterogeneous enhancement following administration of contrast.

MRI

Brain/Paranasal sinuses

CT and MRI through the paranasal sinuses demonstrate a large aggressive mass the epicenter of which appears to be centered in the superior aspect of the nasal cavity.

The mass extends superiorly into the anterior cranial fossa, laterally into the orbit and posteriorly into the sphenoid sinus. The outflow of the sphenoid sinus and left frontal sinus is presumably obstructed as the sinuses are opacified. 

The mass is of soft tissue density, intermediate signal on T1 and T2 weighted images, and demonstrates extensive heterogeneous enhancement following administration of contrast.

The patient went on to have excision of the mass. 

Histology : Sections show a densely cellular high-grade neoplasm displaying small and large areas of necrosis. The tumour is composed of sheets, lobules and broad trabeculae of tumour cells with intervening fibrovascular septa and reactive desmoplastic stroma. Focal formation of Homer Wright rosettes is also noted. Tumour cells have scant cytoplasm and round nuclei with fine chromatin pattern and show nuclear molding. There is focal nuclear pleomorphism. Mitoses and apoptotic bodies are frequent. Occasional foci  display larger neoplastic cells with abundant cytoplasm and larger nuclei. 

The tumor shows wide dissemination within the respiratory mucosa and also infiltrates the adjacent bone. In the orbital region, the tumour invades the connective tissue, skeletal muscles and fat. Foci suggestive of vascular invasion are noted. Tumour cells are strongly immunoreactive for synaptophysin and NSE and weakly positive for chromogranin and S100 protein. Neurofilament immunostaining is negative.

Case Discussion

Location and appearances are typical of olfactory neuroblastoma, which was proven histologically. Differential to consider would be a sinonasal carcinoma and meningioma/hemangiopericytoma.  

Some relevant aspects of olfactory neuroblastoma:

  • 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 the presence of distant metastases
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Case information

rID: 8861
Published: 4th Mar 2010
Last edited: 15th Aug 2020
System: Head & Neck
Inclusion in quiz mode: Included

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