第四脑室内原发性中枢神经系统淋巴瘤1例

2019-08-18 战文建 解哨 晁晓峰 医药前沿

患者,男,61岁,因“头晕伴呕吐半月余”于2017年8月1日入院。既往有银屑病史十余年,外用药物控制。查体见患者神清,精神可,颈软,双侧瞳孔等大等圆,直径约3mm,对光反射灵敏,四肢活动自如,肌力Ⅴ级,肌张力不高,指鼻试验阴性。

1.临床资料
 
患者,男,61岁,因“头晕伴呕吐半月余”于2017年8月1日入院。既往有银屑病史十余年,外用药物控制。查体见患者神清,精神可,颈软,双侧瞳孔等大等圆,直径约3mm,对光反射灵敏,四肢活动自如,肌力Ⅴ级,肌张力不高,指鼻试验阴性。
 
头颅MR平扫+增强检查提示:四脑室后缘见结节影,呈长T1长T2信号,边界欠清,增强后可见病灶均匀明显强化,四脑室受压,脑沟回清晰,中线结构居中(图1.A、B、C、D、E)。胸腹部CT检查未见占位性病变。患者于2017年8月3日在全麻下行开颅肿瘤切除术,术中见肿瘤位于四脑室内,与小脑半球、蚓部、扁桃体粘连,肿瘤软韧不均,色灰白,镜下全切肿瘤,大小约3 cm×2 cm×3 cm。病理(图1.F)及免疫组化:CD20(+),PAX-5(+),CD10(+),BCL-6(+),CD19(+),MUM1(-),CD3(T细胞+),CD5(T细胞+),Ki-67(+约70%),CD30(-),EMA(-),C-Myc(+<40%),Bcl-2(散+),CKpan(-),CD38(+),CD138(-),KAPPA(-),LAMBDA(-),CD43(T细胞+),GFAP(-),NSE(-),S100(-),原位杂交:EBER(-),结合HE切片,为B细胞淋巴瘤,符合弥漫大B细胞淋巴瘤,生发中心起源。



图1 患者术前MR平扫+增强及术后HE切片。
 
患者术后复查头颅MR增强提示四脑室后缘结节强化影(图2.A、B)。一月后患者因头晕伴呕吐症状复现,予头颅MR增强检查提示病灶进展(图2.C、D),住院予三维适形放疗,并行5周期化疗(甲氨蝶呤+替莫唑胺),2018年5月10日患者头颅MR增强检查未见肿瘤复发(图2.E、F)。



图2 患者术后MR增强扫描结果。
 
2.讨论
 
原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)是指起源并仅累及中枢神经系统的非霍其金淋巴瘤,此病恶性程度高、预后差,自然病程约3~6个月,综合治疗后平均生存期约15~45个月。PCNSL可累及大脑、脊髓、眼睛、脑膜和颅神经,但不累及身体其他部位。
 
原发性中枢神经系统淋巴瘤发病率低,占中枢神经系统肿瘤的3%,占所有淋巴瘤的1%。PCNSL在男性患者中发病率高,多于40~60岁间发病,常见于免疫缺陷患者,但在我国此病多发生于免疫缺陷正常患者。脑室内原发性中枢神经系统淋巴瘤(intraventricular primary central nervous system lymphoma,IVPCNSL)是PCNSL中一种罕见的类型,目前也只见于个案报道。
 
IVPCNSL可见于侧脑室和四脑室,可为单发或多发,有些可合并脑实质内病变。临床上单发于四脑室的淋巴瘤更是少见。此病临床表现无特异性,可出现颅高压症状如头痛、恶心呕吐,因位于脑室,可引起梗阻性脑积水。目前脑室内原发性淋巴瘤误诊率高,主要是归因于其临床症状、影像学及实验室检查缺乏特异性表现。
 
总结已报道的PCNSL及IVPCNSL的影像学检查特点,并结合此例,我们发现四脑室内原发性中枢神经系统淋巴瘤的一些特点:(1)在头颅MR平扫+增强检查中表现为长或等T1信号,在T2相上表现为长或等T2信号,注射对比剂后肿瘤一般明显均匀强化;(2)肿瘤一般形态欠规则,可附着于脑室壁爬行性生长,部分可通过脑室孔道向外生长;(3)肿瘤一般与脑干之间留有间隙,很少伴有脑积水发生;(4)实验室检查无特异性,免疫系列及艾滋病检查大多为阴性。
 
第四脑室原发性中枢神经系统淋巴瘤一般需与脑室内肿瘤如脑膜瘤、脉络丛乳头状瘤、室管膜等鉴别:(1)脑膜瘤好发于侧脑室,一般形态较规则,增强后明显强化,女性发病高,而IVPCNSL形态不规则,好发于男性;(2)脉络丛乳头状瘤好发于侧脑室三角区,一般起源于脉络丛,因肿瘤异常分泌脑脊液常合并非梗阻性脑积水,增强扫描强化明显,但局部放大后可见占位颗粒状似桑葚样改变。(3)室管膜瘤一般起源于四脑室底,增强可见蜂窝样强化,可合并脑积水发生。
 
根据欧洲神经肿瘤协会制定的免疫功能正常的原发CNS淋巴瘤循证治疗指南,原发性中枢神经系统淋巴瘤的诊断必须经过病理学诊断,其中免疫组化结果必不可少,以(CD19,CD20,PAX5)、BCL6、MUM1/IRF4和CD10等的B细胞标志应作为主要标记。原发性中枢神经系统淋巴瘤对放疗及化疗均较敏感,在手术或穿刺明确病理后根据病情应采用全脑放疗,然后应用大剂量甲氨蝶呤联合大剂量阿糖胞苷或替莫唑胺进行化疗。另外对于复发难治的PCNSL可考虑采用大剂量化疗联合自体造血干细胞移植。
 
本例患者因头晕伴呕吐等症状入院,并行开颅肿瘤切除术,术后患者头晕及呕吐症状消失,患者好转出院。术后因患者个人原因未及时行放疗,术后一月复查MR提示肿瘤较术前明显扩大,这提示淋巴瘤恶性程度高,生长速度迅速,予全脑放疗后并行5个周期性化疗,定期复查头颅MR提示患者肿瘤体积逐渐缩小并消失。综上,第四脑室原发性中枢神经系统淋巴瘤的影像学诊断缺乏特异性指标,影像学检查误诊率高,手术或立体定向穿刺获取标本是明确本病的直接方法,术后联合放化疗是延长患者生命的有效手段。
 
原始出处:

战文建,解哨,晁晓峰.第四脑室内原发性中枢神经系统淋巴瘤1例并文献复习[J].医药前沿,2018,(21):78-80.

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    2020-05-28 yyj062
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    2019-08-20 tastas

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