Optimum treatment for primary intracranial Ewing sarcoma

Ewing sarcoma (ES) is an aggressive, high-grade neuroectodermal neoplasm that frequently manifests in children and young adults. Although ES without osseous involvement most commonly involves paravertebral regions of the spine, it very rarely presents as a primary intracranial tumor. This report discusses a unique presentation of an adult extraosseous ES arising from the pineal region with extension into the third and fourth ventricles and multiple drop metastases to the spine. This case demonstrates the application of current chemotherapeutic and adjuvant management and offers insight into possible treatment modalities for metastasis in an atypical extraosseous ES involving the brain and spine.