Shuangshoti S, Pitakdamrongwong N, Poneprasert B, Bhavilai D, Kasantikul V
Department of Pathology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
Surg Neurol. 1988 Oct;30(4):298-304. doi: 10.1016/0090-3019(88)90303-5.
A 2-year-old boy had increased intracranial pressure and convulsions because of two extraventricular cysts in the posterior cranial fossa. Releasing the watery cyst contents resulted in disappearance of such clinical manifestations. Immunohistochemically, the epithelial cytoplasm of the cyst was strongly positive for glial fibrillary acidic protein. Electron-microscopically, the cells that lined the cysts had microvilli which were coated with finely granular material to represent "fuzzy" coat, intercellular junctions linking the plasma membranes of adjacent cells, and numerous cytoplasmic glial filaments. The strong positivity for glial fibrillary acidic protein, then, is well correlated with an abundance of the latter. Cilia were also identified. Thus, the cysts were unequivocally neuroepithelial (ependymal) cysts. Only nine cases of neuroepithelial cysts in the posterior cranial fossa, including the current one, have been reported. Three patients were children; six were adults. The age of the patients ranged from 7 months to 60 years. The average age was 21.7 years The sex was known in eight patients with a male to female ratio of 3:5. The pathogenesis of these neuroepithelial cysts was proposed as related to developmental anomalies.
一名2岁男孩因颅后窝两个脑室外囊肿出现颅内压升高和惊厥。排出囊肿内的水样内容物后,这些临床表现消失。免疫组织化学检查显示,囊肿的上皮细胞质中胶质纤维酸性蛋白呈强阳性。电子显微镜检查发现,囊肿内衬细胞有微绒毛,表面覆盖有细颗粒物质,形成“模糊”被膜,相邻细胞的质膜之间有细胞间连接,还有大量细胞质胶质细丝。因此,胶质纤维酸性蛋白的强阳性与后者的大量存在密切相关。还发现了纤毛。因此,这些囊肿明确为神经上皮(室管膜)囊肿。包括本例在内,仅报道了9例颅后窝神经上皮囊肿。3例患者为儿童,6例为成人。患者年龄从7个月到60岁不等,平均年龄为21.7岁。8例患者的性别已知,男女比例为3:5。这些神经上皮囊肿的发病机制被认为与发育异常有关。