1.颅内神经管原肠囊肿(intracranial neurenteric cysts)
又称肠源性囊肿肠囊肿内胚层囊肿等。它起源于胚胎发育前3周内原始神经肠管、脊索、神经管的形成不全,常好发于纵隔、腹部,而中枢神经系统则少见。神经管原肠囊肿其囊壁为薄的半透明囊壁,囊肿壁上皮可为单层或复层扁平、立方、柱状上皮,上皮层与层之间夹杂着分泌黏蛋白的杯状细胞,与消化道肠壁相似因此得名。囊腔外形光滑,其囊内容物性质多样,从均质的清亮液体到较稠的液体,甚至更为浓稠的像胶样囊肿样不透明的物质。Wilkins等根据囊肿壁的组织来源,将其分为3型:Ⅰ型,囊肿壁基底膜上为单层或假复层柱状或立方上皮(有或无绒毛),类似于胃肠上皮(约50%)、呼吸道上皮(17%)或2种上皮混合存在(33%);Ⅱ型,类似于Ⅰ型加上如下组织,即黏液腺、平滑肌、脂肪、软骨、骨、弹力纤维等;Ⅲ型,类似于Ⅰ型加上室管膜或胶质组织作为固有成分,而不是仅仅包围囊肿。颅内神经管原肠囊肿好发于脑干腹侧、桥延交界前方,位于中线或偏中线,少数可位于枕骨大孔、四脑室、鞍区、侧脑室、额叶等。文献报道因含有不等量蛋白或出血,所以信号表现多样。该瘤少血供,出血机制不明,有待进一步探讨。
颅内病灶应与以下病变鉴别:(1)蛛网膜囊肿。多偏侧生长,好发于中颅窝、桥小脑角区,形态规整,多为类圆形,所有序列信号均与脑脊液一样,脑干可受压,但多无明显变形。神经管原肠囊肿多位于中线或偏中线,T1WI多呈等或高信号。(2)表皮样囊肿。好发于桥小脑角、鞍区及其周围区域,有“见缝就钻”的特点,多呈欠均匀T1WI低、T2WI高信号,少数白色表皮样囊肿也呈T1WI高信号,但关键DWI呈不甚均匀的明显高信号,可有效鉴别。(3)皮样囊肿。中线部位含有脂肪的病变,T1WI也呈高信号,但信号多不均匀,抑脂序列呈低信号,其内见脂液平面、脑沟脑池脑室内见到破裂的脂滴则是其较特异征象。(4)桥小脑角区神经鞘瘤可以表现为囊变完全的肿块,形态多呈分叶状,但增强扫描囊壁明显强化,临床症状也可助诊。(5)颅颊裂囊肿。也可有类似T1WI等或高信号、T2WI高信号,但多较小,肿瘤位于垂体前后叶中间,增强扫描呈“爪征”,其内可有不强化的结节影。位于延前池、颅颈交界处等T1、等T2信号的神经管原肠囊肿应与脑膜瘤、脊膜瘤鉴别,平扫可能较相似,但增强扫描较易鉴别。
颅内神经管原肠囊肿边界清晰无水肿,T1WI多呈等或高信号,T2WI多呈高信号,DWI多呈低信号,信号多较均匀,无强化。结合其好发部位及“类脊髓嵌入征”等,多数病例有望术前确诊。

图1A ~ D 患者,女,50 岁。病灶位于桥小脑角池、脚间池及鞍上池,呈不规则分叶状。A. T1WI 呈均匀高信号; B. T2WI 呈均匀高信号; C. DWI 呈较均匀低信号; D. T1WI 抑脂序列仍呈高信号。

图2A~ C 患者,男,63 岁。病灶位于桥延交界前方,分叶状。A. T1WI 呈均匀等信号; B. T2WI 呈均匀等信号; C. DWI 呈均匀等信号。

图3A~ C 患者,女,4岁。病灶位于脑桥腹侧、脚间池,病灶明显挤压嵌入脑干,脑干明显变薄,类似椎管内神经管原肠囊肿的“脊髓嵌入征”。A. T1WI 呈明显低信号; B. T2WI 呈明显高信号; C. 矢状位增强扫描无强化。

REF.白玉贞,牛广明,高阳.颅内神经管原肠囊肿的MRI表现[J].临床放射学杂志,2015,34(12):1885-1888.

Axial plain T1-weighted image of the brain at the level of pons shows a well-defined extraaxial homogenous hyperintense lesion measuring 4.2 by 2.7 centimeters anterior to brain stem and involving bilateral cerebellopontine angle cisterns.

Contrast-enhanced axial (A), and sagittal (B) T1-weighted images of the brain show nonenhancement of the mass and cyst size of 4.2 centimeters in craniocaudal dimension (B).

REF.Arora R, Rani JY, Uppin MS, Ca R. An unusual case of large posterior fossa neurenteric cyst involving bilateral cerebellopontine angle cisterns: report of a rare case and review of literature. Pol J Radiol. 2014 Oct 10;79:356-9. 

A cyst demonstrated on sagittal T1WI (a) and axial T2WI (b). It was an intradural extramedullary well-demarcated lesion occupying the dorsal space of cerebellar vermis. Six years later, the cyst expanded to a lesion attaining signal intensity alterations on T1WI (c) and T2WI (d). A contrast enhancement with gadolinium of a mural nodule was observed in the rostral parts of the cyst (e). T1WI: T1-weighted images; T2WI: T2-weighted images.

A hypointense on T1WI (a) and hyperintense on T2WI (b) multi-lobular cyst in the left cerebellopontine angle. A follow-up MRI ((c), (d)) revealed that some compartments of the lobules were significantly expanded compared with the lesion imaged seven years before ((a), (b)). The signal intensities of expanded compartments were homogeneous hyperintense on T1WI (c) and hypointense on T2WI (d). T1WI: T1-weighted images; T2WI: T2-weighted images; MRI: magnetic resonance imaging.

颅内囊性病变总结(4):神经管原肠囊肿、侧脑室脉络丛囊肿

Watanabe N, Akasaki Y, Fujigasaki J, Mori R, Aizawa D, Ikeuchi S, Murayama Y. Imaging alterations due to squamous metaplasia in intracranial neurenteric cysts: A report of two cases. Neuroradiol J. 2016 Jun;29(3):187-92.

Neurenteric cysts compressing the brainstem. Magnetic resonance imaging demonstrated multiple, nonenhancing, multiolobulated, expanding, and extra-axial recurrent neurenteric cysts compressing the medulla (a). An intraoperative image showing the cysts which were adherent to but separable from the medulla and upper cervical spinal cord (b).
Viaene AN, Brem S. Recurrent neurenteric cysts compressing the brainstem. Surg Neurol Int. 2019 Dec 13;10:245. 

Preoperative MR images of the craniocervical junction. A: The T2-weighted axial image shows a homogeneously hyperintense cyst (arrowhead) anterior to the medulla. B and C: Pre-gadolinium T1-weighted axial and sagittal images show a homogeneously hyperintense cyst that compresses the brain stem at the anterior craniocervical junction. D: The post-gadolinium T1-weighted axial image shows no enhancement of the cystic lesion. E: Diffusion MR image shows a homogeneously hyperintense cyst without diffusion restriction. F: Perfusion MR images reveal partial uptake of the contrast medium in the anterior part of the cystic lesion.

Intraoperative photographs of neurenteric cyst resection using the far-lateral transcondylar approach. A: The right semispinalis captitis muscle was seen, and the dissection of the superior and inferior oblique muscles was performed. B: To secure the vertebral artery, the groove of the vertebral artery was located and lateral partial suboccipital craniotomy was performed. Condylectomy was performed to optimize the view of the anterior brain stem. C: A well-defined capsulated cyst that adheres to the lower cranial nerve and vertebral artery was seen. Posterior bulging of the medulla and cervical cord were seen. D: The lesion was punctured and filled with yellowish viscous fluid. E: The decompressed and lax cyst was completely resected after dissection of the adhesion, which was approximately 10 mm long. F: The premedullary cistern was subsequently secured. The medulla, cervical cord, low cranial nerve, C1 nerve root, and vertebral artery were intact.
Kim WY, Lim J, Cho KG. Anterior Craniocervical Junctional Neurenteric Cyst. Brain Tumor Res Treat. 2021 Oct;9(2):106-110. 

A: An axial T1-weighted magnetic resonance (MR) image with gadolinium enhancement demonstrated a cystic lesion at the left cerebellopontine angle. The wall of a cystic lesion along with the brainstem was slightly enhanced with gadolinium. B: Postoperative T1-weighted MR image showed remarkable shrinkage of the cyst. The hyperintense region indicated a small accumulation of blood inside the cyst cavity (arrowhead). C: The wall of the cyst was lined by a single layer of columnar epithelium (hematoxylin and eosin, 400×). Immunohistochemical analysis showed that the epithelium stained positive for carcinoembryonic antigen (CEA) (D, 200×), epithelium membrane antigen (E, 400×), and cytokeratin (AE1/AE3; F, 400×). Staining for the p53 mutation was negative (H). Periodic acid-Schiff staining demonstrated mucins along the epithelium (G, 200×). The MIB-1 labeling index was almost 0% (I, 400×).

A: Axial magnetic resonance (MR) images demonstrating recurrence of a cystic lesion with a small and slightly enhanced solid portion adjacent to the brainstem (arrow). B: Atypical nuclei and loss of cell polarity were noted (hematoxylin and eosin, 400×). C: Staining for the p53 mutation was weakly positive. D: Immunohistochemical staining with MIB-1 antibody showed approximately 3% nuclear staining in the tumor cells.

Axial magnetic resonance (MR) images revealing significant recurrence of the solid mass with compression of the brain stem (A) and a new gadolinium-enhanced lesion in the right frontal lobe (B). C: Atypical and mitotic nuclei with more prominent than normal nuclei. Pseudostratified nuclei and loss of cell polarity were widely observed (hematoxylin and eosin, 400×). D: Periodic acid-Schiff staining showed strong positivity for mucins. E: Immunohistochemistry for the p53 mutation was positive. F: The MIB-1 labeling index was elevated to 9%.

Fujisawa N, Oya S, Higashi M, Matsui T. Malignant Transformation of a Neurenteric Cyst in the Posterior Fossa Presenting with Intracranial Metastasis: A Case Report and Literature Review. NMC Case Rep J. 2015 Sep 3;2(4):123-127. 

2.脉络丛囊肿(Choroid plexus cyst)
侧脑室脉络丛囊肿又称侧脑室内蛛网膜囊肿脉络膜上皮囊肿,其起源及发病机理尚无定论。目前多认为其为神经上皮起源,与室管膜囊肿统称为神经上皮样囊肿,脉络丛囊肿是最常见的神经上皮样囊肿之一。脉络丛上皮由脑室顶部神经上皮分化而来,和其他间充质一起突入脑室形成脉络丛,在胚胎发育过程中,内衬于原始脑室内的神经上皮发生折叠、内卷或外翻,形成一袋状囊肿凸向脑室内,从而形成囊肿,囊袋颈可离断而使囊肿与脑室分离,囊肿可在脑室内任何部位发展,但以三角区最多见。脉络丛囊肿的囊壁由厚薄不一的疏松结缔组织构成,内衬立方或扁平上皮细胞,具有分泌功能。由于囊液高渗透压使周围组织中液体进入囊肿而逐渐增大,临床主要表现为头痛、头晕、呕吐、视物模糊等颅内压增高征象,个别病例有癫痫发作史。
侧脑室脉络丛囊肿多见于侧脑室三角区其次为侧脑室体部。CT示脑室局限性变形增大,呈圆形或卵圆形,边缘清楚,与脑脊液密度相同,钙化脉络丛受压移位。多数侧脑室脉络丛囊肿MRI上呈等于或接近脑脊液信号,T1WI为均匀低信号,T2WI为均匀高信号,FLAIR T2WI囊液与脑脊液一样被抑制呈低信号。Kjos等将囊肿依MRI信号表现分为3类:(1)低信号型:囊液与脑脊液信号相似;(2)等信号型:囊液含较多量蛋白;(3)高信号型:囊液为胶质或囊内出血。本组病例4例为低信号型,而等信号型或高信号型少见。MRI比CT能更容易显示囊肿的边界和包膜,由于囊壁很薄,囊肿贴近脑室壁处包膜不能显示,而与脑脊液交界处显示概率较高,尤以T2WI效果最佳,成为诊断的重要依据之一。由于囊肿的占位效应,脉络丛受压移位,也是本病的重要征象之一,由于CT对钙化的显示较好,因此观察脉络丛钙化、移位优于MRI,对未钙化的脉络丛观察与MRI一样比较困难,需仔细调整窗宽和窗位。囊肿较大时可使中线结构受压、移位,但周围无脑水肿表现,由于囊肿无血管结构,故囊壁、囊液均无强化。
侧脑室脉络丛囊肿需与以下疾病鉴别:(1)脑室局部增大。原发性少见,常见于脑外伤、脑血管病等引起的局限性脑萎缩,除脑室局部增大外,还伴有周围脑组织密度减低或信号异常,局部脑沟增宽,MRI无囊壁显示,脉络丛无受压、移位;(2)脑室内脑囊虫病。脑室内较大囊虫也可达2~3cm大小,呈薄壁囊肿样改变,常引起室管膜炎,出现脑积水及脑室壁增厚、变形等改变;(3)脑室内皮样囊肿和表皮样囊肿。密度或信号不同于脑脊液,CT值为负值,并可见钙化,MRI信号不均,边界可不规则;(4)胶样囊肿。常见于室间孔部位,引起脑积水,MRI呈短T1、长T2特征表现,不难鉴别。
右侧脑室体部脉络从囊肿
右侧脑室三角部脉络从囊肿

REF.李保卫,赵合保,高立威等.侧脑室脉络丛囊肿的MRI、CT诊断[J].临床放射学杂志,2007(08):757-759.

Preoperative computed tomographic scan demonstrating only asymmetry of the lateral ventricles, with enlargement of the right lateral ventricle.

Preoperative MR images demonstrate a large cystic lesion occupying almost completely the enlarged right lateral ventricle with CSF-like signal intensity and a unilateral right-sided hydrocephalus with deviation of the septum pellucidum to the left. (A) T2-weighted axial image. (B) T1-weighted coronal image.
(A) Intraoperative image reveals whitish wall of cyst obstructing the right foramen of Monro completely. (B) After the midportion of the cyst wall above the foramen of Monro was fenestrated, cystic fluid was expelled.

Gross photographic finding of extracted choroid plexus cyst.

Jeon JH, Lee SW, Ko JK, Choi BG, Cha SH, Song GS, Choi CH. Neuroendoscopic removal of large choroid plexus cyst: a case report. J Korean Med Sci. 2005 Apr;20(2):335-9.