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硬脊膜内髓外双原发性室管膜瘤和脑膜瘤:罕见病例报告及文献复习

Intradural extramedullary double primary ependymoma and meningioma rare condition: Case report and literature review.

作者信息

Yuan Xingyue, Li Ruibo, Liu Qian

机构信息

Department of Pathology, Deyang Peoples' Hospital, Deyang, Sichuan Province, China.

Department of Orthopaedics, Deyang Peoples' Hospital, Deyang, Sichuan Province, China.

出版信息

Medicine (Baltimore). 2025 Jan 10;104(2):e41210. doi: 10.1097/MD.0000000000041210.

Abstract

RATIONALE

Ependymomas are commonly prevalent intramedullary neoplasms in adults, with hardly any cases of exophytic extramedullary ependymoma being reported. Meningiomas, on the contrary, are one of the most common intradural extramedullary (IDEM) tumors. However, the occurrence of both IDEM tumors simultaneously is extremely rare.

PATIENT CONCERNS

A 63-year-old female who presented with pain and numbness in both lower limbs, and symptoms rapidly progressed over the past 5 months.

DIAGNOSIS

Based on the patient's clinical symptoms and imaging features, we conducted pathological examination and genetic testing, ultimately confirming that the patient had IDEM double primary ependymoma and meningioma.

INTERVENTIONS

Surgery was performed to remove double spinal tumors, decompress spinal nerve roots, and perform laminectomy, and she was treated with electrocardiogram monitoring, antibiotics, hemostasis, and antiedema therapy.

OUTCOMES

Histopathology confirmed World Health Organization grade II ependymoma at L2 and World Health Organization grade I meningioma at T12-L1. MYCN amplification and other genetic alterations were absent. Postoperative recovery was favorable, with no recurrence at 6-month follow-up.

LESSONS

This is the first reported case of IDEM double primary ependymoma and meningioma, highlighting the rarity of such cases and the importance of thorough diagnostic workup and surgical excision for IDEM tumors. Genetic analysis adds to the understanding of these rare tumors and guides management strategies.

摘要

理论依据

室管膜瘤是成人常见的髓内肿瘤,几乎没有外生性髓外室管膜瘤的病例报道。相反,脑膜瘤是最常见的硬脊膜内髓外(IDEM)肿瘤之一。然而,两种IDEM肿瘤同时发生极为罕见。

患者情况

一名63岁女性,出现双下肢疼痛和麻木,症状在过去5个月迅速进展。

诊断

根据患者的临床症状和影像学特征,我们进行了病理检查和基因检测,最终确诊患者患有IDEM双原发性室管膜瘤和脑膜瘤。

干预措施

进行手术切除双脊柱肿瘤,减压脊神经根,并进行椎板切除术,同时对她进行心电图监测、抗生素、止血和抗水肿治疗。

结果

组织病理学证实L2节段为世界卫生组织II级室管膜瘤,T12-L1节段为世界卫生组织I级脑膜瘤。无MYCN扩增及其他基因改变。术后恢复良好,6个月随访无复发。

经验教训

这是首例报道的IDEM双原发性室管膜瘤和脑膜瘤病例,突出了此类病例的罕见性以及对IDEM肿瘤进行全面诊断检查和手术切除的重要性。基因分析有助于加深对这些罕见肿瘤的理解并指导管理策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b112/11729158/7ddae46db0d5/medi-104-e41210-g001.jpg

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