Esthesioneuroblastoma

Case contributed by Dr Bruno Di Muzio

Presentation

Epistaxis.

Patient Data

Age: 23
Gender: Female
CT

CT Face

In the right ethmoid air cells and involving also the superior portion of the right nasal cavity there is a soft tissue hypoattenuating mass that extends through the lamina papyracea to the right orbits abutting the medial rectus muscle, and also extends intracranially through the right cribriform plate. No intraconal extension is identified. This lesion shows heterogeneous contrast enhancement. 

MRI

A moderate size lobulated mass is seen centred in the right ethmoid air cells, with intracranial extension through the right cribriform plate and extension into the right orbit. Overall dimensions are 3.7 centimetres SI, 1.8 centimetres transverse and 4.2 centimetres AP. The intracranial component is small with nodular indentation of the right gyrus rectus. Prominent flow voids are seen around this component. Linear dural enhancement seen along the planum sphenoidale and along the lateral floor of the right anterior fossa. The intraorbital component demonstrates lobulated compression of the medial rectus muscle with some linear extension to around the superior oblique muscle. No intraconal extension is identified. High resolution CT scan is recommended to assess the osseous integrity of the medial wall of the right orbit.

Inferior extension involves the middle turbinate muscle. Posterior extension is to the region of the sphenoethmoidal recess and there is lobulated mucosal thickening seen posterior to this within the right sphenoid sinus. There is lobulated mucosal thickening within the right maxillary antrum.

A T2 hyperintense cystic structure is seen superior to the hyoid bone in the root of the tongue measuring 5 x 8 x 7 mm. This likely represents a developmental lesion such as a thyroglossal duct cyst.

Scattered enlarged upper cervical lymph nodes are identified- largest in level 2 bilaterally. Bilateral enlarged retropharyngeal lymph nodes.

Conclusion: Findings compatible with a right-sided esthesioneuroblastoma with a small intra cranial component and extension into the right orbit.

Case Discussion

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

Usually, this tumour 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 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
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Case information

rID: 38353
Case created: 15th Jul 2015
Last edited: 18th May 2017
System: Head & Neck
Tag: rmh
Inclusion in quiz mode: Included

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