无功能性胰腺神经内分泌瘤侵及脾脏一例

2018-10-12 陶奉明 刘爱连 张秋萍 临床放射学杂志

患者男,49岁。近1年消瘦30余斤,在外院门诊行CT检查,发现脾门区占位性病变。1周后入本院进一步诊治。患者高血压病2年余,既往无腹痛、头晕、腹泻、贫血等症状。

患者男,49岁。近1年消瘦30余斤,在外院门诊行CT检查,发现脾门区占位性病变。1周后入本院进一步诊治。患者高血压病2年余,既往无腹痛、头晕、腹泻、贫血等症状。
 
体检:生命体征平稳,皮肤及巩膜无黄染,腹部平坦,质软,未触及肿物,无反跳痛及肌紧张。实验室检查:血常规、肾功、CEA、CA125、CA199、AFP均正常,淀粉酶80U/L,ALT为33U/L,AST为55U/L。
 
MRI表现:MRI检查胰尾部见不规则团块,与脾脏界限不清,紧密相贴,大小约为9.5 cm×7.1 cm,于T1WI呈不均匀低信号(图1),T2WI呈不均匀高信号(图2),T1FSPGR呈低信号,脾脏内见条片状异常信号,边界尚清,于T1WI、T2WI呈稍低信号,T1FSPGR呈等信号。增强扫描,胰尾病灶动脉期,病灶轻度强化,低于正常胰腺(图3),病灶其内可见多根强化血管,脾动脉受包绕;静脉期及平衡期,病灶整体呈中度强化,强化程度略低于正常胰腺(图4、5);脾脏内病灶强化程度与胰尾病灶相似。静脉期冠状位肿块位于胰尾,与脾脏界限不清(图6)。影像诊断:腹膜后巨大占位,考虑胰尾恶性病变侵及脾脏。
 
图1 T1WI:胰尾部肿块呈不均匀低信号,脾脏内病灶呈低信号;图2 T2WI:胰尾部肿块呈稍高信号,脾脏内病灶呈低信号;图3动脉期,胰尾区病灶略强化,强化程度低于正常胰腺,脾脏内病灶轻度强化;
 
图4、5静脉期及平衡期,胰尾区病灶呈中度强化,强化程度略低于胰腺,脾脏内病灶强化程度与胰尾病灶相似
 
图6 静脉期冠状位,肿块位于胰尾,与脾脏界限不清
 
手术及病理:入院13天后,患者行腹膜后肿物及胰体尾、脾脏切除术。术中见肿物位于胰体尾,侵及脾脏与后腹膜,包绕脾动脉,活动欠佳,质硬,直径约为10 cm,大体标本呈灰红色。镜下所见:瘤细胞排列呈索状、段带及片状,瘤细胞立方状,大小形态较一致,核分裂像不易见(图7)。免疫组织化学:Keratin(+)、NES(+)、Cg-A(+)、Syn(+)、Ki-67<5%(+)(图8)。病理诊断:无功能性胰腺神经内分泌瘤侵及脾脏。

图7 HE染色切片(HE×200):瘤细胞排列呈索状、段带及片状,瘤细胞立方状,大小形态较一致,核分裂像不易见;图8 免疫切片:NES(+)、Cg-A(+)、Syn(+)、Ki-67<5%(+)
 
讨论
 
胰腺神经内分泌瘤(nonfunctioning pancreatic endocrine tumors,NFPET)是一组起源于胰腺神经内分泌细胞的肿瘤,约占所有胰腺肿瘤的1%~2%。在流行病学统计中,NFPET发病率从1973年的每10万人中发病率为0.17增长到2007年的每10万人中发病率0.47。临床上,多数NFPET因激素类物质分泌增多有临床表现,被称为功能性NFPET。没有过多内分泌激素导致临床症状或发现激素(如突触素等免疫反应仍为阳性)但不足够引起独特的临床症状,称为无功能性NFPET。
 
NFPET特征性组织学表现为均匀一致的小圆细胞,由于生长方式不同呈现出小梁状、条带状。在免疫组织化学上,Ki-67标记阳性率越高,组织分化越差,本例小于5%诊断为高分化,文献报道NFPET良恶性诊断不能依据免疫组织化学,这与本例相符。NFPET可见于任何年龄,但罕见于儿童,在性别上没有差异。NFPET发病隐匿不引起典型症状和体征,往往因体检或肿块体积大压迫或侵犯毗邻脏器产生症状而就诊,发现时瘤较大,平均约为4~10 cm,其中大部分伴远处转移。肿瘤主要发生于胰腺体尾部,因为胰腺体尾部胰岛细胞分布多,也有文献报道无明显好发部位。
 
House等认为NFPET多数为低度恶性,早期手术治疗效果及预后较其他恶性胰腺肿瘤好,可以根治病变、缓解症状、提高患者远期生存率。近10年国内外对NFPET磁共振表现报道约有十余篇,NFPET为富血供肿瘤,体积较大,增强多数呈不均匀强化或环形强化,而本例呈渐进性强化。结合Tan等与本例影像表现,归纳NFPET磁共振影像特征如下:T1WI为不均匀低信号,T2WI为不均匀高信号,T1FSPGR呈较正常胰腺信号低。增强多表现为不均匀或渐进性强化,因肿瘤体积大,易发中心囊变、坏死。多伴有远处器官或淋巴结转移。
 
本例NFPET发生在胰尾,应主要与以下疾病鉴别:(1)胰腺癌:Noone等研究发现,NFPET表现与胰腺癌相似,应重点区别。胰腺癌位于胰尾时常侵犯脾门,其内可发生坏死液化,但胰腺癌为少血供肿瘤,增强时病灶强化不明显,而周围正常胰腺强化明显,血管根部易受累。(2)腹膜后淋巴瘤:位于胰尾区淋巴瘤表现为边界清楚的多个圆形或类圆形软组密度影或可融合成分叶状团块影,T1WI呈等或稍低信号,略高于肌肉而低于脂肪信号;T2WI上呈高信号,与周围脂肪类似,增强检查呈轻度强化。NFPET临床表现缺乏特异性,很难做出定性诊断,但依据磁共振表现应该考虑NFPET的可能,并且磁共振对软组织分辨率高,更有助于临床决定治疗方案。
 
原始出处:

陶奉明,刘爱连,张秋萍,陈丽华,邓锡佳,李博.无功能性胰腺神经内分泌瘤侵及脾脏一例[J].临床放射学杂志,2018,37(01):169-170.

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    2019-03-29 gwc392
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    2018-10-17 1237db7em74暂无昵称

    学习一下

    0

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