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脊髓弥漫性中线胶质瘤(H3K27改变)行脊髓切除术术后长期生存:病例说明

Long-term survival after cordectomy in a case of spinal cord diffuse midline glioma, H3K27-altered: illustrative case.

作者信息

Sato Daisuke, Takami Hirokazu, Tanaka Shota, Takayanagi Shunsaku, Ikemura Masako, Saito Nobuhito

机构信息

1Department of Neurosurgery, The University of Tokyo Hospital, Tokyo, Japan; and.

2Department of Pathology and Diagnostic Pathology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.

出版信息

J Neurosurg Case Lessons. 2023 Dec 18;6(25). doi: 10.3171/CASE23296.

Abstract

BACKGROUND

Spinal cord diffuse midline glioma, H3K27-altered, is an extremely rare entity with a poor prognosis. However, its optimal treatment remains poorly defined. Although cordectomy was introduced in the early 20th century, its efficacy has been questioned and shrouded behind the scenes.

OBSERVATIONS

A 76-year-old male with recent-onset paraparesis of the lower extremities and paresthesia presented to our outpatient clinic. Magnetic resonance imaging revealed an intra-axial spinal cord tumor extending from T12 to L2. The patient underwent laminectomy and partial tumor resection, and the surgical specimen was histologically diagnosed as a diffuse midline glioma, H3K27-altered. Although standard chemoradiotherapy was implemented, the patient experienced local tumor recurrence 2 years later and underwent cordectomy at T9. The patient was alive at the 4-year follow-up after cordectomy without tumor recurrence. According to the literature, patients with lesions in the lower thoracic cord below T8 achieved a longer survival than those with lesions in the upper thoracic cord above T5.

LESSONS

Cordectomy benefits selected cases of high-grade spinal cord gliomas. Maximal prevention of cerebrospinal fluid dissemination by tumor cells is indisputably important, and tumors located below the lower thoracic spine may be the key to success in establishing a long-term prognosis after cordectomy.

摘要

背景

脊髓弥漫性中线胶质瘤,H3K27改变,是一种极其罕见的疾病,预后较差。然而,其最佳治疗方法仍不明确。尽管脊髓切除术在20世纪初就已引入,但其疗效一直受到质疑且鲜为人知。

观察结果

一名76岁男性因近期出现下肢轻瘫和感觉异常前来我院门诊就诊。磁共振成像显示一个轴内脊髓肿瘤,从T12延伸至L2。患者接受了椎板切除术和部分肿瘤切除术,手术标本经组织学诊断为弥漫性中线胶质瘤,H3K27改变。尽管实施了标准的放化疗,但患者在2年后出现局部肿瘤复发,并在T9进行了脊髓切除术。脊髓切除术后4年随访时患者存活,无肿瘤复发。根据文献,T8以下胸段脊髓病变的患者比T5以上胸段脊髓病变的患者生存期更长。

经验教训

脊髓切除术对部分高级别脊髓胶质瘤病例有益。最大程度地防止肿瘤细胞播散至脑脊液无疑至关重要,位于下胸椎以下的肿瘤可能是脊髓切除术后获得长期预后成功的关键。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e4d/10732319/e6c6cd6f8686/CASE23296f1.jpg

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