继发性颅内恶性纤维组织细胞瘤一例

2019-06-22 韦小白 吴学勇 中国肿瘤临床与康复

病例资料:男,25岁,因“左腋窝纤维组织细胞瘤术后3个月,复发1月”入院。2016年10月因咳嗽咯痰至当地医院就诊,肺部CT提示右上肺占位,行右上肺肿瘤切除术。术后病理诊断:深部透明细胞为主的良性纤维组织细胞瘤。2017年2月发现左腋窝肿块,肺部CT提示右肺术后改变,左侧腋窝团块影。2017年2月15日行左腋窝包块扩大切除术,术后病理诊断:梭形细胞肿瘤,结合病史考虑为纤维组织细胞瘤,未行放化疗治疗

恶性纤维组织细胞瘤(malignant fibrous histiocytoma,MFH)是主要由成纤维细胞和组织细胞组成的恶性肿瘤,多发生于深部组织,以四肢(尤以下肢)和腹膜后常见。常转移至肺部,转移至颅脑少见。而颅内MFH的临床和影像表现缺乏特异性,加上发生率极低,故极易误诊。
 
病例资料:男,25岁,因“左腋窝纤维组织细胞瘤术后3个月,复发1月”入院。2016年10月因咳嗽咯痰至当地医院就诊,肺部CT提示右上肺占位,行右上肺肿瘤切除术。术后病理诊断:深部透明细胞为主的良性纤维组织细胞瘤。2017年2月发现左腋窝肿块,肺部CT提示右肺术后改变,左侧腋窝团块影。2017年2月15日行左腋窝包块扩大切除术,术后病理诊断:梭形细胞肿瘤,结合病史考虑为纤维组织细胞瘤,未行放化疗治疗。
 
2017年3月现左侧肢体麻木和乏力进行性加重,至当地医院就诊,头颅CT提示右侧额叶占位。PET/CT提示:右侧额叶略高密度灶,伴周边水肿带,脱氧葡萄糖(fluorodeoxyglucose,FDG)代谢不均匀,异常增高,左侧腋窝肿大淋巴结,FDG代谢异常增高,考虑转移瘤可能。行颅脑占位切除术,术后病理镜下:梭形瘤细胞束状排列,核异形,见核分裂像,坏死。
 
免疫组化:瘤细胞:GFAP(脑+)Olig2(-)IDH1(-)P53(少+)ATRX(-)MIB-1(5%)SMA(-)KP1(+)S100(灶+)CK(-)Vim(+),提示右颞顶纤维组织细胞瘤伴坏死,考虑恶性,术后未行放化疗治疗。2017年5月左侧腋窝下再现肿块,迅速增大,直径6×5 cm,位置固定,表皮稍红。至我院行异环磷酰胺/美斯钠+吡喃阿霉素+氮烯咪胺(MAID方案)化疗1个疗程。疗效不佳。复查头颅MR考虑右侧额叶占位,符合转移瘤术后复发表现(图1)。2周后左腋下肿块迅速增大至10×8 cm(图2)。


图1A:TIWI等/低信号;B:T2WI高信号;C:磁共振成像液体衰减反转恢复序列等高信号;D:DWI弥散受限(DWI高信号);E:轴位TIWIC+:右侧额叶增强后呈环状厚壁强化,周围见大片水肿信号;F:冠状位TIWIC+:额顶叶强化灶,临近脑膜增厚强化


图2A:患者左腋下肿块;B:肺部CT增强提示左腋下不均匀强化灶
 
2017年6月再次行左腋下肿块切除术,术后病理:左前胸壁梭形细胞间叶源性或纤维组织细胞源性恶性肿瘤,直径10×8 cm。全自动免疫组化:CK(-),Vimentin(+),SMA(-),Desmin(-),S-100(少量散在阳性),CD34(-),CD68(-),CD117(-),CD99(-),EMA(-),P53(-),KI67(+,20%)。2017年7月行二次颅内占位切除术。
 
该病例肿块呈恶性生长态势,发展迅速。首次脑瘤切除术后40天,发现右颞顶叶肿块再次复发,左腋下肿块术后45天原位再发。由于原有肺部肿块病理诊断为良性,首次左腋下肿块术后病理仅提示梭形细胞肿瘤,无免疫组化,病理诊断不明确。后神经系统症状逐渐加重,经MR检查颅内恶性肿瘤,术后病理证实为(右颞顶)恶性纤维组织细胞瘤。结合PET/CT,左腋下淋巴肿大,诊断考虑继发性恶性纤维组织细胞瘤。
 
第2次左腋下肿块术后病理提示:梭形细胞间叶源性或纤维组织细胞源性恶性肿瘤,证实了该诊断,符合恶性纤维组织细胞瘤恶性度高、转移快、易复发和预后差等特点。颅内影像学特点:由于恶性纤维组织细胞瘤发病率低,关于其颅内转移瘤的影像学特点,近10年来国内仅有少数报道,如将颅内恶性纤维组织细胞瘤的影像学特点分为脑外表现和脑内表现。
 
MRI表现为T1WI等或略低,T2WI不均匀高信号,增强扫描均可见明显强化,脑外占位常累及脑膜及颅骨,边界清晰,瘤周不伴或仅有轻度水肿。关于颅内MFH磁共振扩散加权成像(diffusion weighted imaging,DWI)影像学特点的相关报道极少见,目前有少数作者指出,部分四肢恶性纤维组织细胞瘤DWI呈高信号,且仅多见于囊性改变和内部多个分隔特点者。除MR特点外,有个案报道表明,MRS检查中明显升高的Lip峰可能有利于MFH诊断。DWI序列是目前唯一能够检测活体组织水分子扩散运动的无创方法。不过关于颅内恶性纤维组织细胞瘤的DWI成像特点的报道较少。
 
本例患者DWI高信号,与其他脑实质转移瘤DWI低信号有明显不同。临床上,从DWI呈高信号影的角度出发,MFH需与急性脑梗死和淋巴瘤等其他疾病鉴别。
 
1.急性脑梗死:DWI高信号伴相应的ADC值低信号。TIWI早期皮质肿胀和低信号,灰白质边界不清,T2WI高信号(起病12~24h形成皮质水肿),起病后2~3周可能转为正常,磁共振成像液体衰减反转恢复序列(fluid-attenuated inversion recovery,FLAIR)脑实质高信号表现,起病后6h,其他序列正常,血栓形成的血管常呈高信号,对早期梗死更敏感,DWI弥散受限(DWI高信号)。
 
2.原发性中枢神经系统淋巴瘤:基底神经节和室周白质强化病变。TIWI等/低信号,T2WI均质性等/低信号,FLAIR均质性等/低信号,DWI弥散受限(DWI高信号),TIWIC+显著均质的增强或中心型坏死和均质增强的周围强化,无增强非常罕见。
 
3.多形性胶质母细胞瘤:肿块中间为坏死区,外周可有不规则的厚强化边缘,为肿瘤组织,侵及脑膜罕见。TIWI不规则等/低信号白质肿块,T2WI不均匀高信号肿块,伴有肿瘤侵润/血管性水肿,可见坏死、囊变、出血、液体/碎片平面及留空影(新生血管生成),肿瘤实际范围常远远超过影像学异常信号之外,FLAIR不均匀高信号肿块,伴肿瘤侵润/血管性水肿,DWI无弥散受限(DWI低信号),TIWIC+典型表现为中央坏死区周围有不规则的厚边缘强化,可有多种强化类型:实性、环形、结节样和斑片状。
 
4.脑实质转移瘤:灰白质交界区离散的强化病变。TIWI等/低信号,高信号少见,如黑色素瘤,T2WI信号的变化取决于细胞密度、核浆比率及有无出血。临床常见肺癌直肠癌乳腺癌等转移瘤T2WI呈高信号,FLAIR常为中度高信号肿块,伴显著高信号的交界区水肿,DWI通常无弥散受限(DWI低信号),TIWIC+几乎所有转移瘤均存在强化。本案例DWI高信号,与其他脑实质转移瘤DWI低信号显著不同。
 
5.脑脓肿:脓肿发展的不同阶段,影像学表现不同。包膜期早期:边界清楚、薄壁环形增强。环形增强病灶DWI高信号;T2环形低信号,伴环绕水肿。TIWI脑炎期早期:边缘不整,混杂性的低信号/等信号病变;脑炎期晚期:中心低信号,边缘等信号/略高信号;包膜期早期:相对于白质,边缘呈等/高信号,相对于脑脊液,中心呈高信号;包膜期晚期:空腔缩小、囊壁增厚。T2WI脑炎期早期:边缘不清的高信号病变;脑炎期晚期:中心高信号,周围低信号,水肿呈高信号;包膜期早期:周边低信号;包膜期晚期:水肿和占位效应减轻。DWI脑炎和脓肿信号增高。TIWIC+脑炎期早期:斑片状增强;脑炎期晚期:显著不规则周边增强;包膜期早期:边界清楚,薄壁的周边增强;包膜期晚期:空腔塌陷,囊壁增厚。
 
MFH对单纯的放疗或化疗效果不佳,目前多数学者主张根治性手术辅以放疗和化疗的综合措施。本例患者化疗效果不佳,目前服用伊马替尼靶向药物,临床效果尚待进一步观察。由于原发MFH及转移性颅内MFH病例较少,关于其影像学诊断特点有待继续收集病例,深入研究。
 
原始出处:

韦小白,吴学勇.继发性颅内恶性纤维组织细胞瘤一例[J].中国肿瘤临床与康复,2018,25(06):762-763.

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    2019-11-14 xzw113
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  3. [GetPortalCommentsPageByObjectIdResponse(id=1952680, encodeId=5282195268081, content=<a href='/topic/show?id=a37ce8961a1' target=_blank style='color:#2F92EE;'>#继发性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=78961, encryptionId=a37ce8961a1, topicName=继发性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=2317301, createdName=xzw113, createdTime=Thu Nov 14 01:23:00 CST 2019, time=2019-11-14, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1775721, encodeId=86721e75721a9, content=<a href='/topic/show?id=f59f5292ea7' target=_blank style='color:#2F92EE;'>#恶性纤维组织细胞瘤#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=36, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=52927, encryptionId=f59f5292ea7, topicName=恶性纤维组织细胞瘤)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=789538794376, createdName=appleandpeer, createdTime=Tue Jul 30 17:23:00 CST 2019, time=2019-07-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1724495, encodeId=a3331e24495c6, content=<a href='/topic/show?id=78ece8958b2' target=_blank style='color:#2F92EE;'>#继发#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=23, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=78958, encryptionId=78ece8958b2, topicName=继发)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=499d33645837, createdName=feifers, createdTime=Tue Dec 17 16:23:00 CST 2019, time=2019-12-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=368319, encodeId=fa00368319f1, content=666, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cdnapi.center.medsci.cn/medsci/head/2019/01/04/2540fb84decb81d47a7f2868070e6cbd.jpg, createdBy=ed3f2205793, createdName=百草, createdTime=Tue Jun 25 07:49:31 CST 2019, time=2019-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1475996, encodeId=d97414e5996fb, content=<a href='/topic/show?id=2bcee797732' target=_blank style='color:#2F92EE;'>#细胞瘤#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=35, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=77977, encryptionId=2bcee797732, topicName=细胞瘤)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=bf5e7252474, createdName=spoonycyy, createdTime=Mon Jun 24 14:23:00 CST 2019, time=2019-06-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=368186, encodeId=a4ce3681869e, content=谢谢了,学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=50, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=30331616752, createdName=txqjm, createdTime=Sun Jun 23 07:25:00 CST 2019, time=2019-06-23, status=1, ipAttribution=)]
    2019-12-17 feifers
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    2019-06-25 百草

    666

    0

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    2019-06-23 txqjm

    谢谢了,学习

    0

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