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Ewing sarcoma – sinonasal

Case contributed by Anthony Nuñez
Diagnosis almost certain

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

Case Discussion

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

Findings were consistent with Ewing sarcoma/peripheral primitive neuroectodermal tumors (pPNET) confirmed on histology.

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