Primary intradural Extraosseous Ewing’s sarcoma of the cauda equina: A case report and literature review

IF 1.5 4区 医学 Q4 CLINICAL NEUROLOGY
Manel Krouma , Kaissar Farah , Anis Choucha , Romain Appay , Florence Duffaud , Stephane Fuentes , Henry Dufour
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Abstract

Intradural Extraosseous Ewing sarcoma (IEES) is an infrequent occurrence. We report a case of a 66-year-old male who presented with a 2-month history of low back pain and bilateral S1 sciatica, with acute sphincter dysfunction. Imaging studies revealed an intradural extramedullary lesion in the cauda equina spanning from level L4 to S1. The patient underwent partial removal of the intradural lesion. Histopathological examination showed the presence of small round cells, which were consistent with Ewing Sarcoma. The patient was then treated with targeted radiation therapy and chemotherapy.

The rarity of IEES in this specific location underscores the significance of evaluating and managing patients with intradural spinal tumors with careful consideration of this diagnosis. To further investigate this condition, we conducted a thorough review of the literature on IEES involving the lumbar spine and cauda equina. Our analysis revealed that patients with this condition frequently exhibit rapidly progressive neurological symptoms likely attributed to hemorrhagic transformation. This characteristic may serve as a distinguishing factor from other lesion types, particularly benign ones. Our study provides a comprehensive summary that can offer direction for clinical management in comparable uncommon and novel cases.

马尾原发性硬膜外尤文氏肉瘤:病例报告和文献综述。
硬膜外尤文肉瘤(IEES)并不常见。我们报告了一例 66 岁男性的病例,他因腰痛和双侧 S1 坐骨神经痛 2 个月,并伴有急性括约肌功能障碍。影像学检查显示,马尾从 L4 到 S1 有硬膜外病变。患者接受了硬膜内病灶部分切除术。组织病理学检查显示存在小圆形细胞,与尤文肉瘤一致。在这一特殊部位出现 IEES 的罕见性凸显了在评估和治疗脊柱硬膜内肿瘤患者时仔细考虑这一诊断的重要性。为了进一步研究这种情况,我们对涉及腰椎和马尾的 IEES 文献进行了全面回顾。我们的分析表明,这种病症的患者经常表现出快速进展的神经症状,很可能是出血性转化所致。这一特征可能是区别于其他病变类型(尤其是良性病变)的一个因素。我们的研究提供了一个全面的总结,可为临床治疗可比的不常见和新型病例提供方向。
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来源期刊
Neurochirurgie
Neurochirurgie 医学-临床神经学
CiteScore
2.70
自引率
6.20%
发文量
100
审稿时长
29 days
期刊介绍: Neurochirurgie publishes articles on treatment, teaching and research, neurosurgery training and the professional aspects of our discipline, and also the history and progress of neurosurgery. It focuses on pathologies of the head, spine and central and peripheral nervous systems and their vascularization. All aspects of the specialty are dealt with: trauma, tumor, degenerative disease, infection, vascular pathology, and radiosurgery, and pediatrics. Transversal studies are also welcome: neuroanatomy, neurophysiology, neurology, neuropediatrics, psychiatry, neuropsychology, physical medicine and neurologic rehabilitation, neuro-anesthesia, neurologic intensive care, neuroradiology, functional exploration, neuropathology, neuro-ophthalmology, otoneurology, maxillofacial surgery, neuro-endocrinology and spine surgery. Technical and methodological aspects are also taken onboard: diagnostic and therapeutic techniques, methods for assessing results, epidemiology, surgical, interventional and radiological techniques, simulations and pathophysiological hypotheses, and educational tools. The editorial board may refuse submissions that fail to meet the journal''s aims and scope; such studies will not be peer-reviewed, and the editor in chief will promptly inform the corresponding author, so as not to delay submission to a more suitable journal. With a view to attracting an international audience of both readers and writers, Neurochirurgie especially welcomes articles in English, and gives priority to original studies. Other kinds of article - reviews, case reports, technical notes and meta-analyses - are equally published. Every year, a special edition is dedicated to the topic selected by the French Society of Neurosurgery for its annual report.
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