Brain Tumor Res Treat.
2026 Apr;14(2):102-108. 10.14791/btrt.2025.0035.
Primary Intradural Extramedullary Ewing Sarcoma of the Thoracic Spine With Leptomeningeal and Brain Metastases: A Case Report and Literature Review
- Affiliations
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- 1Department of Neurology, Medical Faculty of Universitas Muslim Indonesia, Makassar, South Sulawesi, Indonesia
- 2Division of Neuro-Oncology, Neurology Department of Medical Faculty of Hasanuddin University, Makassar, South Sulawesi, Indonesia
- 3Division of Hematology and Medical Oncology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
- 4Departments of Pathology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
- 5Departments of Radiation Oncology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
- 6Departments of Neurosurgery, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
- KMID: 2580973
- DOI: http://doi.org/10.14791/btrt.2025.0035
Abstract
- A 43-year-old woman presented with bilateral lower extremity weakness due to an intradural extramedullary spinal cord tumor. Surgery revealed Ewing sarcoma, a rare presentation known as primary intradural extramedullary Ewing sarcoma (PIEES). Despite initial treatment with radiation and chemotherapy, tumor recurrence occurred after 17 months. Further interventions included additional surgery, radiation, and chemotherapy. The disease progressed to leptomeningeal metastases along the spinal cord, prompting various treatments including targeted spinal radiation and systemic therapies. Brain metastases subsequently developed, necessitating whole-brain radiation and intrathecal chemotherapy. This case highlights the aggressive nature of PIEES, its potential for widespread leptomeningeal metastasis, and the challenges in its management, underscoring the need for multidisciplinary approaches in treating this rare and aggressive malignancy.
