Primary Intradural Extramedullary Ewing Sarcoma of the Thoracic Spine With Leptomeningeal and Brain Metastases: A Case Report and Literature Review
Abstrak
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.
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.
Kata Kunci
Primary intradural extramedullary Ewing sarcoma; Ewing sarcoma; Leptomeningeal metastases.