Yeoh G P, Bale P M, de Silva M
Institute of Pathology, Royal Alexandra Hospital for Children, Sydney, Australia.
Pediatr Pathol. 1989;9(5):531-49. doi: 10.3109/15513818909026912.
Twenty two nasal cerebral heterotopias were compared with 11 nasal encephaloceles. No histological feature was found that would allow a communication with the brain to be confidently identified or excluded. Even laminated cerebral cortex with neurones and ependymal canals, suggestive of encephalocele, were found in heterotopias. Distinction required radiological and surgical evidence. However, CT scan could be misleading, in one infant suggesting a cribriform plate defect when none was found at craniotomy. Three children had multiple extracranial glial lesions, two with both heterotopia and encephalocele in the same patient. In a few older children it was extremely difficult to identify brain tissue because of marked replacement by fibrous tissue (up to 95%), leading to one misdiagnosis as fibroma, and considerable fibrosis occurred also in five of six recurrences and in a longstanding small encephalocele. In two heterotopias, cellularity in places approached that of low-grade neoplastic glioma. One nasopharyngeal heterotopia contained multiple mesenchymal tissues suggestive of teratoma. Two midline nasopharyngeal encephaloceles showed adjacent epithelium, possibly vestiges of Rathke's pouch.
对22例鼻脑异位与11例鼻膨出进行了比较。未发现能够可靠地识别或排除与脑相通的组织学特征。甚至在鼻脑异位中也发现了具有神经元和室管膜管的分层大脑皮质，提示脑膨出。鉴别需要影像学和手术证据。然而，CT扫描可能会产生误导，在一名婴儿中，CT扫描提示筛板缺损，但开颅手术时并未发现。3例儿童有多处颅外胶质病变，2例患者同时存在鼻脑异位和脑膨出。在一些大龄儿童中，由于纤维组织大量替代（高达95%），很难识别脑组织，导致1例误诊为纤维瘤，在6例复发病例中的5例以及1例长期存在的小型脑膨出中也出现了大量纤维化。在2例鼻脑异位中，局部细胞密度接近低级别肿瘤性胶质瘤。1例鼻咽部鼻脑异位包含多个提示畸胎瘤的间充质组织。2例中线鼻咽部脑膨出显示相邻上皮，可能是拉克囊的遗迹。
