椎管内髓细胞肉瘤1例

2019-01-28 白世琦 李万颖 耿任 中国肿瘤临床

髓细胞肉瘤(myeloid sarcoma,MS)是一种发生在骨髓外的,由伴有或不伴有成熟迹象的髓系原始细胞构成的肿块。MS 可发生于身体任何位置,发生于椎管内的非常罕见。本文报道1例椎管内髓细胞肉瘤。

髓细胞肉瘤(myeloid sarcoma,MS)是一种发生在骨髓外的,由伴有或不伴有成熟迹象的髓系原始细胞构成的肿块。MS 可发生于身体任何位置,发生于椎管内的非常罕见。本文报道1例椎管内髓细胞肉瘤。
 
患者男,22岁,2017年7月因“进行性双下肢麻木无力10天伴尿便障碍3天”就诊于吉林大学第一医院神经肿瘤外科,既往无血液病及感染病史。查体:体温36.8℃,血压126/75 mmHg,心率80次/分。无贫血貌,全身浅表淋巴结无肿大。双上肢肌力5级,肌张力正常,双下肢肌力0级,肌张力减低,双上肢腱反射正常对称引出,双下肢腱反射未引出。T6平面以下痛觉消失。双下肢深感觉消失。双侧Babinski征、Chaddock征阴性,无项强、克氏征阴性、大小便失禁。
 
MRI示:胸4~11椎体水平硬膜囊外侧见多发结节状长T1短T2异常信号,压脂像呈高及低信号,增强扫描异常强化,相应水平脊髓受压向前移位(图1)。
 


图1 患者胸椎核磁影像。胸4-11椎体水平硬膜囊外侧见多发结节状异常信号,增强扫描异常强化。A:T1WI,B:T2WI,C:增强扫描
 
血常规:白细胞(WBC)3.61×109/L,中性粒细胞百分比(NE)37%,淋巴细胞百分比(LY)54%,单核细胞百分比(Mo)9%,嗜酸性粒细胞百分比(Eo)为0.28%,嗜碱性粒细胞百分比(BA)0,中性粒细胞绝对值(NE)1.33×109/L,淋巴细胞绝对值(LY)1.94×109/L,单核细胞绝对值(Mo)0.33×109/L,嗜酸性粒细胞绝对值(Eo)0.01×109/L,嗜碱性粒细胞绝对值(BA)0.00×109/L,红细胞(RBC)4.68×1012/L,血红蛋白(HGB)137 g/L,血小板(PLT)149×109/L。脑脊液流式细胞检测未见异常细胞。
 
患者椎管内占位诊断明确,且脊髓压迫症状明显,病情进展迅速,手术指征明确,于全麻下行椎管内肿物切除术。术中打开T4~7椎管,见椎管内肿物为淡褐色、鱼肉状、质地较韧、血运差,贴敷于硬脊膜外侧,似炎性机化组织。切除全部肿物送检病理。术后患者下肢肌力、肌张力未见明显改变,右侧感觉平面下降至T6水平。术后病理结果回报:送检组织内可见髓细胞肉瘤。
 
免疫组织化学结果:Ki-67(+80%),MPO(+),Vimentin(-),CK-pan(-),S-100(-),CD20(-),CD3(-),SALL4(-),CgA(-),Syn(-),CD99(-),MyoD1(-),Fli-1(+),Desmin(-),CD68(-),CD7(-),ALK(-),CD30(-),CD43(+),CD34(-),CD4(-),CD56(+),Lysozyme(-),CD117(+)(图2)。分子病理结果:B细胞基因重排(-),免疫球蛋白IGH、IGκ链未发现基因重排克隆。综上,可诊断为髓细胞肉瘤。建议患者行骨穿、染色体检查等,并继续相应治疗,但患者及家属拒绝。
 


图2 术后病理结果。送检组织内可见髓细胞肉瘤,免疫组织化学示MPO(+)。A:H&E×200;B:IHC×200
 
小结
 
MS多继发于急、慢性髓系白血病,骨髓增生异常综合征等恶性血液病,但也有少数患者没有前驱恶性白血病或者MS早于恶性血液病典型血液学改变之前出现,称为原发性MS,易出现误诊。本例患者虽然血常规检查不支持白血病,但患者未行骨髓穿刺,因此无法明确患者属于哪一类型MS。
 
MS可发生于身体任何位置,因此临床症状各不尽相同,与肿瘤位置及大小密切相关。椎管内MS主要表现为神经根疼痛,压迫平面以下感觉减退,肌力下降甚至截瘫,可伴有大小便失禁。目前MS的发病机制尚不明确,据报道可能与白血病细胞归巢信号在髓外的异常表达有关。
 
MS的微观组成最常见的是成髓细胞、嗜中性粒细胞和嗜中性粒细胞前体的混合成分。MS 的诊断往往需要免疫组织化学结果的验证,包括MPO、CD68/KP1、CD43、CD117、CD99等,其中MPO在MS诊断中的特异性和敏感性较高。另外约55%的病例染色体发生畸变,所以可以通过FISH或细胞遗传学来证实诊断。椎管内MS治疗困难,患者病情进展迅速,预后较差。绝大多数原发性MS会在诊断后数月内进展为急性髓系白血病,而进展到急性髓系白血病的病例很少能生存1年以上。
 
尽管椎管内MS可以通过手术、局部或全身放疗、系统化疗、骨髓移植等方法进行治疗,但预后仍然很差。有报道称接受系统性化疗比接受局部手术或放疗的患者,进展为白血病的时间更长,但椎管内MS,尤其是原发性MS往往诊断较为困难,手术可以帮助诊断而且可以解除压迫症状,这也给治疗方法的选择造成了困难。近年来,同种异体造血干细胞移植对MS的治疗带来了新的希望。目前尚缺乏MS大样本量治疗案例,需要进一步的临床研究,MS的诊断及治疗方法仍需要我们不断地探索。
 
原始出处:

白世琦,李万颖,耿任,曹赢坤,李蕴潜.椎管内髓细胞肉瘤1例[J].中国肿瘤临床,2018,45(02):107-108.

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    2019-02-09 bugit
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    2019-02-09 jyzxjiangqin

    椎管内髓细胞肉瘤。

    0

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    2019-01-31 jyzxjiangqin

    椎管内髓细胞肉瘤。

    0

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    2019-01-30 chengjn
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    2019-01-29 1dd8a7c5m95(暂无匿称)

    认真学习,不断进步,把经验分享给同好。点赞了!

    0

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