Pediatric Cranial Ewing Sarcoma: A Rare Case Treated with Multimodal Management

Background:

Ewing sarcoma (ES) is a highly aggressive bone tumor predominantly affecting children and adolescents, most commonly arising in the long bones and pelvis. Primary involvement of the cranial bones is exceedingly rare, accounting for only 1% of all ES cases, with the frontal bone being an uncommon site.

Case Presentation:

We report the case of a 4-year-old child who presented with a progressive, non-tender swelling over the left frontal region. Radiological investigations revealed a 5.5 cm lesion at the left frontal convexity, suggestive of a sarcomatous skull lesion. The patient underwent left fronto-parieto-temporal craniotomy and near-total excision of the extradural mass, followed by cranioplasty. Histopathological and immunohistochemical analyses confirmed the diagnosis of Ewing sarcoma of the skull. The patient received multimodal therapy, including VDC/IE chemotherapy and adjuvant radiotherapy. Despite developing neutropenia and grade II skin toxicity during treatment, the patient achieved complete radiological response on follow-up PET scan and remains neurologically intact.

Conclusion:

Primary cranial Ewing sarcoma, though rare, should be considered in pediatric patients presenting with persistent skull masses. Early diagnosis, complete surgical excision, and protocol-based chemo-radiotherapy can lead to favorable outcomes. This case underscores the need for increased awareness and collaborative research to improve treatment strategies for this rare entity.