Skull-base Ewing sarcoma with multifocal extracranial metastases.

Area of Science:

• Neuro-oncology
• Skeletal oncology
• Rare tumors

Background:

• Ewing sarcoma (ES) is a rare bone and soft tissue malignancy.
• Intracranial ES is exceptionally uncommon, with skull-base involvement being anecdotal.
• Early diagnosis and management are crucial for improving patient outcomes.

Observation:

• A 29-year-old man presented with rapidly progressive ophthalmoplegia.
• An infiltrative lesion involving the sphenoid sinus, sella, and clivus was identified.
• Histological examination suggested Ewing sarcoma.

Findings:

• The patient developed paraparesis two weeks after initiating adjuvant therapy.
• Imaging revealed thoracic extradural lesions and widespread vertebral and pulmonary metastases.
• This represents the first reported case of primary intracranial skull-base ES with disseminated extracranial disease.

Implications:

• This case highlights the potential for aggressive behavior and rapid dissemination of intracranial ES.
• It underscores the importance of considering rare diagnoses in the differential for skull-base lesions.
• Further research into the biology and treatment of intracranial ES is warranted.