原发性脊柱硬膜内髓外尤文氏肉瘤-一种罕见的侵袭性肿瘤

Rav Tej Bathala , Jishnu N Nair , H. Mohamd Naleer , Vivek Visweswaran , Ganesh Krishnamurthy
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摘要

硬膜内髓外尤文氏肉瘤(IDEM)是一种罕见的脊柱肿瘤,需要多学科联合治疗,并且有很高的复发和转移率。本病例报告描述了一名28岁男性患者,他有两个月的背痛病史,并突然加重和急性发作,迅速进展的下肢无力和脐下麻木,并伴有肠和膀胱失禁。脊柱MRI显示D11 - D12椎体水平有清晰均匀增强的IDEM,病变将脊髓推向右侧。患者接受椎板切除术和病变切除,组织病理学证实为尤文氏肉瘤。根据多学科团队的建议,他接受了辅助化疗和放疗,在12个月的随访中,他能够独立行走并在最小的支持下进行日常活动。本病例强调了早期诊断和多学科治疗对改善硬膜内髓外尤文氏肉瘤预后的重要性。鉴于这种病变的罕见性,需要进一步的研究来规范其管理。本报告还回顾了硬膜内髓外尤文氏肉瘤的现有文献,重点介绍了临床特征、影像学表现、治疗方案和结果。
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Primary Intradural Extramedullary Ewing’s Sarcoma of spine – A rare and aggressive tumour

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.

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1.00
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0.00%
发文量
236
审稿时长
15 weeks
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