双语病例——Rathke裂囊肿
翻译老师：凌一童 山东省日照市人民医院
朗读老师：李嘉颖 浙江省中医院
审校老师：姜春雷 青岛市第九人民医院
History and CT images
History: A 53-year-old woman with no significant past medical history presented with new-onset generalized headache. She underwent an unenhanced CT. An axial image and coronal image are shown below. Click to enlarge.
病史和CT图像
病史：一名53岁女性出现新发的广泛性头痛，既往无特殊意义的病史。她接受了CT平扫检查。轴位图像和冠状位图像如下图所示。
MR images
The patient subsequently underwent an MRI of the brain. Select images from coronal T1-weighted precontrast, coronal T1-weighted postcontrast, sagittal T1-weighted precontrast, sagittal T1-weighted postcontrast, coronal T2-weighted, and axial fluid-attenuated inversion-recovery (FLAIR) sequences are provided.
MR图像
病人随后接受了脑部核磁共振检查。选取冠状位T1预对比、冠状位T1增强、矢状位T1预对比、矢状位T1增强、冠状位T2和轴位T2 FLAIR序列的图像。
Findings and diagnosis
Findings
· CT: Circumscribed, hypo- to isoattenuating mass in the sella with minimal suprasellar extension but no contact with the optic chiasm
· MRI:
· T1: mildly hyperintense relative to brain parenchyma
· T2: hyperintense to brain parenchyma but less intense than cerebrospinal fluid (CSF)
· Enhancement: no internal enhancement
· FLAIR: no suppression
· Possible T1 hypointense peripheral nodule
Differential diagnosis
· Pituitary adenoma
· Rathke cleft cyst
· Craniopharyngioma
· Meningioma
· Aneurysm
· Teratoma
Diagnosis: Rathke cleft cyst
结果和诊断
结果
· CT: 鞍区局限性、低至等密度肿块，鞍上轻微延伸，但未累及视交叉。
· MRI:
· T1: 比脑实质信号略高
· T2: 比脑实质信号高但比脑脊液的信号低
· 增强: 无内部强化
· FLAIR: 无抑制
· 外周结节T1可能呈低信号
鉴别诊断
· 垂体腺瘤
· Rathke裂囊肿
· 颅咽管瘤
· 脑膜瘤
· 动脉瘤
· 畸胎瘤
诊断: Rathke裂囊肿
Key points
关键点
Rathke cleft cyst
Pathophysiology
· During embryologic development, Rathke pouch arises as an outpouching of the oral aspect of the developing alimentary tract’s ectoderm.
· The anterior pituitary develops from the pars distalis and pars tuberalis, both derived from the anterior wall of Rathke pouch.
· The pars intermedia arises from the posterior wall of Rathke pouch and separates the anterior and posterior pituitary.
· Rathke cleft can persist if space between the anterior pituitary and pars intermedia does not obliterate.
· Cysts can develop within the persistent cleft.
· Cysts are lined by columnar or cuboidal epithelium, often with cilia and goblet cells.
· Cysts may contain an intracystic nodule of cellular debris.
Epidemiology
· Common; present in up to one-third of adults
· More common in adults and females
Rathke裂囊肿
病理生理学
· 在胚胎发育过程中，Rathke囊作为发育中消化道外胚层的口腔开口
· 垂体前叶由远侧部和结节部发育而来，两者均来自 Rathke 囊的前壁。
· 中间部起源于 Rathke 囊的后壁，将垂体前部和后部分开。如果垂体前叶和中间部之间的间隙没有消失，Rathke 裂可能会持续存在。
· 囊肿可在残存裂隙内发育形成。
· 囊肿壁由柱状或立方上皮排列而成，通常带有纤毛和杯状细胞。
· 囊肿可能含有由细胞碎片构成的囊内结节。
流行病学
· 常见，可见于1/3的成年人
· 在成年人和女性中更常见
Clinical presentation
· Usually incidental and asymptomatic
· Headaches
· Changes in vision with suprasellar origin or extension and compression of the optic chiasm
· Hypopituitarism
临床症状
· 通常偶发或无症状
· 头痛
· 鞍上起源或延伸压迫视交叉可出现视力改变
· 垂体功能减退
Imaging features
· CT:
· Circumscribed, low-attenuation mass in the sella or suprasellar
· Internal calcification is absent, but thin peripheral calcifications are uncommonly seen
· No internal enhancement, but the wall may minimally enhance
· MRI:
· T1: hyper- or hypointense, depending on the presence of proteinaceous material
· T2: more commonly hyperintense, but can be hypointense
· No internal enhancement, but the wall may minimally enhance
· Intracystic nodules have been described as being specific for Rathke cleft cyst. They are typically T1 hyperintense and T2 hypointense relative to the rest of the cyst, but signal characteristics can vary -- in this case, there was a small peripheral area of relative hypointensity on T1 pre- and postcontrast images, possibly representing a nodule.
影像学特征
· CT:
· 蝶鞍或鞍上局限的低密度肿块
· 内部无钙化，周围薄层钙化不常见
· 内部无强化，但内壁可轻微强化
· MRI:
· T1:高信号或低信号，取决于蛋白质物质的存在
· T2:常为高信号，但也可呈低信号
· 内部无强化，但囊壁可轻微强化
· 囊内结节被描述为Rathke 囊肿的特征性表现。相对于囊肿的其他部位，通常呈T1高信号和T2低信号，但信号特征可能不同。在本例中，在 T1 对比增强前后图像上有一个周边区域相对小低信号灶，可能代表一个小结节。
Differential diagnosis
The differential for sellar and suprasellar masses is broad and includes the following:
· Pituitary adenoma
· Rathke cleft cyst
· Craniopharyngioma
· Meningioma
· Aneurysm
· Teratoma
Treatment
· Usually requires no treatment. If large (> 10 mm) but still asymptomatic, some practitioners will follow the cyst with MRI.
· If symptomatic, transnasal transsphenoidal drainage of the cyst is preferred.
· Craniotomy is rarely required and generally avoided.
鉴别诊断
蝶鞍和鞍上的鉴别很广泛，包括以下内容:
· 垂体腺瘤
· Rathke裂囊肿
· 颅咽管瘤
· 脑膜瘤
· 动脉瘤
· 畸胎瘤
治疗
· 通常不需要治疗。如果囊肿较大( > 10 mm )，但仍无症状，一些医生会通过MRI对囊肿进行随访。
· 如有症状，首选经鼻蝶窦囊肿引流。
· 很少需要开颅手术，一般需避免。