Intraspinal Extradural Myxopapillary Ependymoma of the Sacrum: A Case Report

Prashant Punia, A. Chugh, S. Gotecha
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Abstract

Background Data:Myxopapillary ependymoma (MPE) is a low-grade variant of ependymoma that typically occurs in conus medullaris and filum terminale of the spinal cord. Only rarely do these tumors present at an extradural location in the sacrococcygeal region and even rarer is the development of these extradural tumors inside the spinal canal. The authors report a case of an intraspinal extradural MPE of the sacrum with relevant literature review on salient features and management. Study Design: Case Report. Purpose: To report a rare entity at an uncommon location. Case Report: A 12-year-old male child presented with chief complaints of low backache and radiation of pain in both lower limbs for six months. The patient also had a history of straining during micturition for one month. On examination, he was found to have mild weakness of bilateral ankle plantar flexion and knee flexion (grade 3). Ankle jerks were absent and there was evidence of sacral hypoaesthesia. Intraoperatively sacral lamina was found to be thinned out. Laminectomy was performed at L5 and sacrum, an extradural, fleshy lesion extending from L5 to S5 was identified, and gross total resection was achieved. There was no intradural expansion of the tumor. Results: Patient had an uneventful course and was discharged home on day 4. Conclusion: Intraspinal extradural MPE is a rare entity and should be kept in mind while diagnosing a midline lower back lesion. Treatment consists of gross total excision wherever possible coupled with radiotherapy. These tumors tend to recur locally and systemically and, thus, patients should be followed up periodically. (2020ESJ226)
骶脊膜内硬膜外粘液状乳头状管膜瘤1例
背景资料:黏液乳头状室管膜瘤(MPE)是一种低级别的室管膜瘤,通常发生在脊髓圆锥和脊髓终丝。这些肿瘤很少出现在骶尾骨区的硬膜外位置,更罕见的是这些硬膜外肿瘤在椎管内的发展。作者报告一例骶骨椎管内硬膜外MPE,并对其显著特征和治疗进行了相关文献综述。研究设计:病例报告。目的:报告一个罕见的实体在一个不寻常的位置。病例报告:一名12岁男童,主诉为腰痛及双下肢放射性疼痛6个月。患者也有一个月的排尿紧张史。检查发现患者双侧踝关节、足底屈曲和膝关节屈曲轻度无力(3级)。踝关节无抽搐,骶骨感觉减退。术中发现骶椎板变薄。在L5和骶骨处行椎板切除术,发现从L5延伸到S5的硬膜外肉质病变,并实现了大体全切除。肿瘤未见硬膜内扩张。结果:患者疗程顺利,第4天出院。结论:椎管内硬膜外MPE是一种罕见的病变,在诊断下背部中线病变时应注意。治疗包括尽可能的全切除和放射治疗。这些肿瘤往往局部和全身复发,因此,患者应定期随访。(2020 esj226)
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