Olfactory neuroblastoma (esthesioneuroblastoma)

Case contributed by Frank Gaillard , 4 Mar 2010
Diagnosis certain
Changed by Bruno Di Muzio, 15 Jul 2015

Updates to Case Attributes

Presentation was added:
Rhinorrhoea and episodes of epistaxis.
Age was set to 50-year-old.
Gender was set to Male.
Body was changed:

Location and appearances are typical forof an olfactory neuroblastoma, which was proven histologically.

Some relevant aspects of this tumour:

  • peaks in young adult patients (~2nd decade) and another peak in the 5th to 6th decades
  • nasal stuffiness and rhinorrhoea 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 tumour 
  • 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
  • -<p>Location and appearances are typical for an<a href="/articles/olfactory-neuroblastoma" title="Olfactory neuroblastoma"> </a>olfactory neuroblastoma which was proven histologically.</p>
  • +<p>Location and appearances are typical of an <a href="/articles/olfactory-neuroblastoma">olfactory neuroblastoma</a>, which was proven histologically.</p><p>Some relevant aspects of this tumour:</p><ul>
  • +<li>peaks in young adult patients (~2<sup>nd</sup> decade) and another peak in the 5<sup>th</sup> to 6<sup>th</sup> decades</li>
  • +<li>nasal stuffiness and rhinorrhoea or epistaxis are also referred as primary symptoms </li>
  • +<li>arises from the basal layer of the olfactory epithelium in the superior recess of the nasal cavity</li>
  • +<li>slow growing tumour </li>
  • +<li>tends to destroy surrounding bone, and can extend in any direction</li>
  • +<li>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 </li>
  • +<li>contrast enhancement is often marked in both CT and MRI studies</li>
  • +<li>treatment usually involves combined chemotherapy and/or radiotherapy with surgical excision</li>
  • +<li>prognosis is significantly affected by presence of distant metastases</li>
  • +</ul>
Diagnostic Certainty was set to .

Updates to Study Attributes

Caption was added:
CT Paransal sinuses
Images Changes:

Image CT (bone window) ( update )

Perspective was set to Coronal.

Image CT (C+ arterial phase) ( update )

Perspective was set to Axial.

Image CT (C+ delayed) ( update )

Perspective was set to Axial.

Image 3 CT (bone window) ( update )

Position was set to .

Updates to Study Attributes

Caption was added:
MRI Brain/Paranasal sinuses
Images Changes:

Image MRI (T1) ( update )

Perspective was set to Sagittal.

Image MRI (T1) ( update )

Perspective was set to Axial.

Image MRI (T1 C+ fat sat) ( update )

Perspective was set to Coronal.

Image MRI (T2) ( update )

Perspective was set to Axial.

Image MRI (T1) ( update )

Perspective was set to Coronal.

Image MRI (T2) ( update )

Perspective was set to Coronal.

Image 2 MRI (T1 C+ fat sat) ( update )

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Image 3 MRI (T1 C+ fat sat) ( update )

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Image 4 MRI (T1) ( update )

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Image 5 MRI (T1) ( update )

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Image 6 MRI (T2) ( update )

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Image 7 MRI (T2) ( update )

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Updates to Freetext Attributes

Description was changed:

The patient went on to have excision of the mass.&nbsp;

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 &nbsp;display larger neoplastic cells with abundant cytoplasm and larger nuclei.&nbsp;

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.

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