Abstract
Background Calvarial Ewing tumor is a relatively rare differential among bony neoplasms. We
present our experience of managing primary calvarial Ewing sarcoma (EWS), highlighting
their clinical and radiological findings.
Method In a retrospective analysis, we evaluated our 12-year database for pathologically
proven EWS. A literature search was conducted for the comparative presentation and
update on the management and outcome.
Result From January 2008 to December 2020, we managed eight patients (male:female = 5:3;
age range 6 months to 19 years, mean 11.5 years) harboring primary calvarial EWS.
All cases underwent wide local excision; two patients required intradural tumor resection,
while one required rotation flap for scalp reconstruction. Mean hospital stay was
8 days. All patients received adjuvant chemo- and radiotherapy. Three patients remained
asymptomatic at 5 years of follow-up, while two patients died.
Conclusion Primary calvarial EWS is a rare entity. It usually affects patients in the first
two decades of life. These tumors can be purely intracranial, causing raised intracranial
pressure symptoms, which may exhibit rapidly enlarging subgaleal tumors with only
cosmetic deformities or symptoms of both. Radical excision followed by adjuvant therapy
may offer a favorable long-term outcome.
Keywords
calvaria - primary - Ewing - radiology
