{"title":"儿童内源性脑干表皮样囊肿。","authors":"Tybault Hollanders, Sarah Hendrickx, Edward Baert","doi":"10.25259/SNI_198_2025","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Epidermoid cysts are slow-growing, rare congenital lesions. They are most seen in the cerebellopontine angle, fourth ventricle, or parasellar regions. Patients typically become symptomatic between 20- and 40 years of age. We present a rare case of a symptomatic intrinsic brainstem epidermoid cyst in a 12-year-old girl with atypical radiological features.</p><p><strong>Case description: </strong>A 12-year-old girl presented with progressive gait disturbances, disequilibrium, diplopia due to right-sided abducens paresis, left-sided facial paresis (HB grade II), headaches, nausea, and vomiting over the past 6 months. Magnetic resonance imaging (MRI) revealed an intrinsic lesion of the brainstem not present on MRI 4 years prior. A microsurgical gross total resection, including resection of cyst wall, was performed. The pathology report diagnosed the lesion as an epidermoid cyst. The patient improved significantly after resection.</p><p><strong>Conclusion: </strong>This pathology represents <1% of all intracranial tumors, and with only 21 documented pediatric intrinsic brainstem cases in medical literature, very rarely has an intrinsic brainstem location. We detail the medical history, work-up, surgical management, and postoperative outcomes, contributing to the limited body of knowledge regarding this exceptional entity.</p>","PeriodicalId":94217,"journal":{"name":"Surgical neurology international","volume":"16 ","pages":"290"},"PeriodicalIF":0.0000,"publicationDate":"2025-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12361696/pdf/","citationCount":"0","resultStr":"{\"title\":\"Intrinsic brainstem epidermoid cyst in childhood.\",\"authors\":\"Tybault Hollanders, Sarah Hendrickx, Edward Baert\",\"doi\":\"10.25259/SNI_198_2025\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Epidermoid cysts are slow-growing, rare congenital lesions. They are most seen in the cerebellopontine angle, fourth ventricle, or parasellar regions. Patients typically become symptomatic between 20- and 40 years of age. We present a rare case of a symptomatic intrinsic brainstem epidermoid cyst in a 12-year-old girl with atypical radiological features.</p><p><strong>Case description: </strong>A 12-year-old girl presented with progressive gait disturbances, disequilibrium, diplopia due to right-sided abducens paresis, left-sided facial paresis (HB grade II), headaches, nausea, and vomiting over the past 6 months. Magnetic resonance imaging (MRI) revealed an intrinsic lesion of the brainstem not present on MRI 4 years prior. A microsurgical gross total resection, including resection of cyst wall, was performed. The pathology report diagnosed the lesion as an epidermoid cyst. The patient improved significantly after resection.</p><p><strong>Conclusion: </strong>This pathology represents <1% of all intracranial tumors, and with only 21 documented pediatric intrinsic brainstem cases in medical literature, very rarely has an intrinsic brainstem location. We detail the medical history, work-up, surgical management, and postoperative outcomes, contributing to the limited body of knowledge regarding this exceptional entity.</p>\",\"PeriodicalId\":94217,\"journal\":{\"name\":\"Surgical neurology international\",\"volume\":\"16 \",\"pages\":\"290\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-07-18\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12361696/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Surgical neurology international\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.25259/SNI_198_2025\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Surgical neurology international","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.25259/SNI_198_2025","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/1/1 0:00:00","PubModel":"eCollection","JCR":"","JCRName":"","Score":null,"Total":0}
Background: Epidermoid cysts are slow-growing, rare congenital lesions. They are most seen in the cerebellopontine angle, fourth ventricle, or parasellar regions. Patients typically become symptomatic between 20- and 40 years of age. We present a rare case of a symptomatic intrinsic brainstem epidermoid cyst in a 12-year-old girl with atypical radiological features.
Case description: A 12-year-old girl presented with progressive gait disturbances, disequilibrium, diplopia due to right-sided abducens paresis, left-sided facial paresis (HB grade II), headaches, nausea, and vomiting over the past 6 months. Magnetic resonance imaging (MRI) revealed an intrinsic lesion of the brainstem not present on MRI 4 years prior. A microsurgical gross total resection, including resection of cyst wall, was performed. The pathology report diagnosed the lesion as an epidermoid cyst. The patient improved significantly after resection.
Conclusion: This pathology represents <1% of all intracranial tumors, and with only 21 documented pediatric intrinsic brainstem cases in medical literature, very rarely has an intrinsic brainstem location. We detail the medical history, work-up, surgical management, and postoperative outcomes, contributing to the limited body of knowledge regarding this exceptional entity.