Primary Intradural Extramedullary Ewing’s Sarcoma of spine – A rare and aggressive tumour

Abstract

Intradural extramedullary Ewing's sarcoma(IDEM) is a rare form of spinal tumor that requires a multidisciplinary approach and has high rates of recurrence and metastasis. This case report describes a 28-year-old male who presented with two months history of back pain and with sudden exacerbation and acute onset rapidly progressing lower limb weakness and numbness below the umbilicus with bowel and bladder incontinence. MRI spine showed a well-defined homogeneously enhancing IDEM at D11 – D12 vertebral level with the lesion pushing the spinal cord to the right side. The patient underwent laminectomy and excision of the lesion, and histopathology confirmed Ewing's sarcoma. He received adjuvant chemotherapy and radiotherapy based on multidisciplinary team advice, and at 12 months follow-up, he was able to walk on his own and carry out his daily activities with minimal support. This case emphasizes the importance of early diagnosis and multidisciplinary management in improving the prognosis of intradural extramedullary Ewing's sarcoma. Given the rarity of this lesion, further research is needed to standardize its management. This report also includes a review of the existing literature on intradural extramedullary Ewing's sarcoma, highlighting the clinical features, radiological findings, treatment options, and outcomes.