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Ewing sarcoma – sinonasal

Case contributed by Dr Anthony Nuñez

Presentation

Patient with headache, visual problems and facial swelling.

Patient Data

Age: 20 years
Gender: Male
MRI

There is an extensive and voluminous soft tissue mass with intermediate signal in T2-weighted imaging and low signal in T1, that involves posterior nasal cavity, paranasal sinuses (sphenoid, posterior ethmoidal cells, left maxillary sinus) and skull base. Foci of susceptibility are scattered throughout the mass in GRE sequence compatible with hemosiderin deposits. Also, diffusion restriction is demonstrated in DWI and ADC map. 

The lesion extends to the anterior cranial fossa, middle cranial fossa, left orbit, palate, pterygopalatine fossa and infratemporal fossa.  

Left proptosis.

Retention material is seen in the left frontal sinus and sphenoidal sinus.

CT

There are bony destructive and remodeling changes on computed tomography.

Case Discussion

Ewing’s sarcoma family of tumors are derived from pluripotent neural crest cells. These tumors are common in the second decade of life. In the craniofacial region, the commonest site is mandible followed by the maxilla 1.

Findings were consistent with a Ewing's sarcoma/Peripheral primitive neuroectodermal tumors (pPNET) (confirmed on histology).

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Case information

rID: 67755
Published: 29th Jun 2019
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included
Institution: University Institute CEMIC

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