成人不典型鞍区毛细胞型星形细胞瘤1例

2019-10-11 谢民 武志 雒以诚 丁涛 国际神经病学神经外科学杂志

患者男,30岁。因“视力进行性下降1年,行走不稳半年”于2017年12月1日入院。眼科检查:右眼视力0.08,左眼指数20CM,眼底视乳头水肿,边界消失,视野检查无法配合完成;双侧轮替、指鼻及跟膝胫试验欠稳准,余无阳性体征。

1.病例资料
 
患者男,30岁。因“视力进行性下降1年,行走不稳半年”于2017年12月1日入院。眼科检查:右眼视力0.08,左眼指数20CM,眼底视乳头水肿,边界消失,视野检查无法配合完成;双侧轮替、指鼻及跟膝胫试验欠稳准,余无阳性体征。
 
入院CT示鞍区扩大,蝶鞍及鞍上葫芦状囊实性肿块影,向鞍上池生长,初诊考虑侵袭性垂体腺瘤(图1a)。次日行MRI检查示鞍上池形态不规则团块状混杂信号,向三脑室生长,向下压迫垂体窝,视交叉受压,正常视交叉结构消失,外周与双侧颈内动脉紧邻,鞍区结构紊乱,肿瘤内部出现“花环样”强化,余无强化,双侧脑室积水(图1b、c、d)。入院后在全麻下行双额冠状切口右额开颅颅底纵裂入路,术中见视交叉下方紫褐色肿瘤组织,质地软,血供较丰富,边界不清,向鞍上池及脚间池生长,肿瘤与视交叉、下丘脑粘连紧密,无法判断肿瘤起源。病理结果示:毛细胞型星形细胞瘤,WHOⅠ级(图1e)。术后患者视力稍有改善,出现尿崩、电解质紊乱、高热,垂体激素复查示T3、T4及皮质醇水平减低,术后2周MRI复查肿瘤基本全切(图1f)。住院1月余好转出院,建议患者行局部放疗,院外继续口服小剂量激素及优甲乐,继续随访。





图1 a.术前CT鞍区扩大,鞍内囊实性肿块影,密度等或低;b.术前MRI病灶最外层及中心呈长T2信号,呈其内部呈不规则等T2信号;c、d.MRI增强见肿瘤内部花环样强化,周围及中心无强化;e.病理示瘤组织无纤维包膜,瘤实质多由Rosenthal纤维和丰富血管组织构成,瘤内见嗜酸性小体,瘤细胞排列疏密不均,核分裂像少见;f.术后2周MRI增强示肿瘤基本全切。
 
2.讨论
 
2016年WHO将毛细胞星形细胞瘤(pilocytic astrocytoma,PA)的恶性程度定位Ⅰ级,其主要见于儿童的星形细胞瘤(约占80%~85%),而在成人星形细胞瘤中少见(约占7%~25%)。毛细胞型星形细胞瘤可发生在所有神经轴,好发部位包括:①小脑(小脑星形细胞瘤),②大脑半球,③丘脑和基底节,④脑干(背外脑干胶质瘤),⑤视路胶质瘤(视交叉/下丘脑/视神经)。脊髓的毛细胞型星形细胞瘤罕见,下丘脑、丘脑和脑干大的病变突入脑室内时很难确定它们的原发部位。发生在不同部位的PA表现不同,可呈实性、囊实性病灶,发生在小脑半球的肿瘤边界清楚,多为囊性或含有较大的囊变区,少数为实性。
 
鞍区PA多起源于视交叉、下丘脑及三脑室底部,此部位的星形细胞瘤主要为毛细胞型星形细胞瘤,约占60%,弥漫纤维型星形细胞瘤(恶性程度为Ⅱ级)约占40%。发生在鞍区毛细胞型星形细胞瘤,多为实性边界清楚的肿块,但毛细胞型星形细胞瘤病理上为良性肿瘤,肿瘤内含水分较多并有较多的小囊变,肿瘤实性部分信号较均匀,可见多发微小囊变,增强后实性部分强化均匀或不均匀,囊变区可强化或不强化,MRI对本病的术前诊断有重要参考价值。
 
本例类似鞍区肿瘤,向三脑室及丘脑生长,向下压迫垂体窝,视交叉受压,鞍区MR信号紊乱,MRI增强见肿瘤内部“花环样”强化,周围及中心无强化,在诊断上较困难。需与鞍上颅咽管瘤、侵袭性垂体腺瘤、生殖细胞瘤等鉴别诊断,而当肿瘤内部出现钙化、囊变、坏死甚至卒中时常不易诊断。确诊需要病理检查,显微镜下以Rosenthal纤维和(或)嗜酸颗粒小体为特征。手术治疗为首选,鞍区PA手术难度大,尽量全切肿瘤,术后需严格控制水、电解质平衡,必要时行激素替代治疗,身体状况允许尽早行术后外放疗,定期复查,整体预后较好。
 
原始出处:

谢民,武志,雒以诚,丁涛,杨刚,郑茂华.成人不典型鞍区毛细胞型星形细胞瘤1例[J].国际神经病学神经外科学杂志,2018,45(04):408-409.

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