大脑镰海绵状血管瘤1例

2020-05-07 安鹏 张晨颖 张银霞 医学影像学杂志

患者男性,60岁。无明显诱因出现左侧脸部麻木两年余,患者1周前因手部外伤入院行全身检查,实验室及体格检查未见明显异常。MRI表现:左侧额部大脑前纵裂旁可见椭圆形稍长T1,等T2信号影,大小约3.5 c

患者男性,60岁。无明显诱因出现左侧脸部麻木两年余,患者1周前因手部外伤入院行全身检查,实验室及体格检查未见明显异常。MRI表现:左侧额部大脑前纵裂旁可见椭圆形稍长T1,等T2信号影,大小约3.5 cm×3.1 cm×4.1 cm,边界清晰,增强扫描呈明显不均匀强化,邻近脑膜增厚、强化,左侧脑室前角略受压,中线结构局部右偏(图1,2A,2B)。

 1588373688416985.png

图1A~1C瘤体内可见增粗强化血管影;T1WI呈稍低信号;T2WI信号不均匀,以等信号为主

 1588373701614796.png

图2A,2BT1WI增强示肿瘤明显不均匀强化;冠状位T1WI增强示病变与大脑镰关系密切

手术及病理:行纵裂颅内占位病变切除术,术中可见肿瘤位于左侧大脑镰旁,与大脑镰关系密切,瘤体内血供丰富,外观呈鲜红色,质地略韧,肿瘤与周围脑组织轻度粘连。术后组织病理学检查:瘤组织由大量形状不规则的血管窦状腔隙构成,管腔大小不等,内皮分化成熟(图2c)。免疫组化:A4:EMA(-);Vimentin(+);PR(-);S-100(-);GFAP(-);STAT6(+);Ki-67(-)。诊断海绵状血管瘤。

 1588373719261651.png

图2C镜下可见瘤组织由不规则的血管窦状腔隙构成,内皮分化成熟(HE,×100)

讨论

脑外型海绵状血管瘤(extracerebral cavernous hemangiomas,ECHs)又称硬膜型海绵状血管瘤,是一种少见的隐匿性脑血管畸形,仅占所有颅内血管畸形的0.4%~2%,临床症状多表现为颅内占位效应所致的头痛、复视和颅神经麻痹。脑外型海绵状血管瘤的生长部位多与静脉窦有关,最常见于颅中窝海绵窦区,少数可位于大脑凸面、小脑幕及颈静脉孔区等,发生于大脑镰的病变十分罕见,国内未见相关个案报道。

本例病变的主要影像学特点如下:1)病灶为单发实质性肿块,边界清晰,肿块内未见钙化;2)CTA显示病灶内可见增粗强化血管影;3)MRI呈稍长T1,等T2信号表现,T2WI肿瘤内可见斑点状低信号影,增强扫描病灶明显不均匀强化,邻近脑膜增厚强化。ECHs在T2WI多表现为高信号,而本例病变T2WI呈等信号,与之前报道有所不同,结合病理结果,这可能与肿瘤富含间质成分、胶原纤维有关。同时,Kaqa等研究认为,病灶内的血管腔及血栓可引起T2WI点状或条状低信号影,此表现与本例病变相符。

另外,刘翔等通过观察12例中颅窝外脑外型海绵状血管瘤发现,血管造影病灶多见肿瘤样染色;同时,Kaqa等回顾以往文献也指出,血管造影可见中动脉至晚期静脉期微弱的肿瘤样染色,此征象鉴别ECHs具有一定价值。

本例病变需与脑膜瘤、血管周细胞瘤相鉴别。1)脑膜瘤:常见于中年女性,形态规则,边界清楚,MRI呈等T1、等T2信号,增强后病灶强化明显,多数可见“脑膜尾”征;梁奕等认为,脑膜瘤ADC值偏低与海绵状血管瘤的鉴别具有一定特异性,此外,脑膜瘤的MRS表现为Cho峰升高,可作为鉴别诊断的补充手段;2)血管周细胞瘤:常见于年轻女性,多呈分叶状,窄基底与硬膜相连,MRI信号多不均匀,常见流空血管影,瘤周占位效应明显,同时,肿瘤具有侵袭性,常破坏邻近骨质。

原始出处:

安鹏,张晨颖,张银霞,高思佳.大脑镰海绵状血管瘤1例[J].医学影像学杂志,2019,29(02):251+256.

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    2020-05-09 cooco

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