Isolated CNS vasculitis: unusual presentation of relapsed Ewing sarcoma

Area of Science:

• Neurology
• Pediatric Oncology
• Rheumatology

Background:

• Ewing sarcoma is a rare bone cancer primarily affecting children and young adults.
• Central nervous system (CNS) involvement in Ewing sarcoma is uncommon and often associated with poor prognosis.
• Expressive dysphasia can be a symptom of various neurological conditions.

Observation:

• A 12-year-old boy presented with expressive dysphasia following treatment for Ewing sarcoma.
• Magnetic Resonance Angiography (MRA) and cerebral angiography revealed CNS vasculitis.
• Extensive rheumatologic investigations did not reveal an underlying autoimmune or inflammatory disease.

Findings:

• Neurological recovery was achieved with prednisone treatment, suggesting an inflammatory or autoimmune process.
• Restaging scans initially showed no evidence of recurrent Ewing sarcoma.
• Recurrent, metastatic Ewing sarcoma was confirmed via biopsy four months later, indicating the vasculitis was a paraneoplastic or presenting sign.

Implications:

• This case underscores the importance of considering CNS vasculitis in pediatric patients with neurological deficits, especially those with a history of Ewing sarcoma.
• Isolated CNS vasculitis can be an early, albeit rare, manifestation of occult or recurrent Ewing sarcoma.
• The findings suggest a potential link between Ewing sarcoma and CNS vasculitis, warranting further investigation into the underlying mechanisms.