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中枢神经系统富含淋巴浆细胞的脑膜瘤:1 例不常见病例报告。

Lymphoplasmacyte-rich meningioma in the central nervous system: An unusual case report.

机构信息

Department of Neurosurgery, West China Hospital, West China Medical School, Sichuan University, Chengdu, PR China.

Department of Neurosurgery, the second People's Hospital of Yibin, Yibin, Sichuan Province, China.

出版信息

Medicine (Baltimore). 2021 Dec 30;100(52):e27991. doi: 10.1097/MD.0000000000027991.

Abstract

RATIONALE

Lymphoplasmacyte-rich meningioma (LPRM) is a rare meningioma characterized by significant infiltration of plasma cells and lymphocytes, and changes in the ratio of meningeal epithelial components. According to the World Health Organization, tumors of the central nervous system are classified as grade I tumors.

PATIENT CONCERNS

A 44-year-old man presented to our department with complaints of limb weakness accompanied by hand numbness. Half a month before admission, the patient's limb weakness worsened and he could not walk and raise his hands, with limb sensory disturbance and incontinence.

DIAGNOSIS

Magnetic resonance imaging of the head and cervical spinal cord showed a diffuse extramedullary mass creeping on the tentorium and skull base meninges along the clivus down to the sixth cervical spinal meninges. The cervical spinal cord was enveloped and pressed (Fig. 1A-C). Postoperative histopathological examination showed meningothelial areas admixed with lymphocytes and plasma cells (Fig. 2D-H), indicating that the mass was a LPRM.

INTERVENTION

Suboccipital craniotomy, C1 laminectomy, and C2-C6 laminoplasty were performed for this patient, and postsurgical pathology showed that the tumor was a LPRM with large amounts of lymphocytes and plasma cells.

OUTCOME

After 2 weeks of active treatment, the patient died of worsening pneumonia.

LESSONS

LPRM is a rare variant of meningioma, and it is more unusual that the lesions involve the intracranial dura mater and the entire cervical spinal meninges. So far, surgical resection has been the main treatment for LPRM, but according to its own characteristics of lymphoplasmacyte-rich, immunotherapy may become a new treatment option.

摘要

背景

淋巴浆细胞丰富型脑膜瘤(LPRM)是一种罕见的脑膜瘤,其特征为浆细胞和淋巴细胞大量浸润,并伴有脑膜上皮成分比例的改变。根据世界卫生组织的分类,中枢神经系统肿瘤被归类为 I 级肿瘤。

患者关注

一名 44 岁男性因四肢无力伴手麻就诊于我科。入院前半个月,患者四肢无力加重,无法行走和抬手,伴有四肢感觉障碍和尿失禁。

诊断

头颅和颈髓磁共振成像显示弥漫性髓外肿块沿小脑幕和颅底脑膜爬行,累及斜坡至颈 6 脊髓脑膜。颈髓被包绕并受压(图 1A-C)。术后组织病理学检查显示脑膜内皮区混杂有淋巴细胞和浆细胞(图 2D-H),提示该肿块为 LPRM。

干预措施

该患者接受了枕下开颅术、C1 椎板切除术和 C2-C6 椎板成形术,术后病理显示肿瘤为 LPRM,大量淋巴细胞和浆细胞浸润。

结果

经过 2 周的积极治疗,患者因肺炎恶化而死亡。

教训

LPRM 是脑膜瘤的一种罕见变异,病变累及颅内硬脑膜和整个颈段脊髓脑膜更为罕见。到目前为止,手术切除一直是 LPRM 的主要治疗方法,但根据其富含淋巴浆细胞的自身特点,免疫疗法可能成为一种新的治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/05a5/8718218/b800ddf4e3a4/medi-100-e27991-g001.jpg

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