{"title":"小儿轴外髓母细胞瘤表现为阻塞性脑积水和扁桃体突出:临床表现和治疗挑战。","authors":"Linyi Zhu, Amer Helal, Xenia Doorenbosch","doi":"10.1136/bcr-2024-263710","DOIUrl":null,"url":null,"abstract":"<p><p>Medulloblastoma is the most common paediatric tumour presenting in the posterior fossa. Typically, they are found midline and intra-axially located. Medulloblastomas presenting in an extra-axial location, however, are rare, and of those, they are usually found in the cerebellopontine angle. We present a case of an extra-axial medulloblastoma projecting from the lateral cerebellar hemisphere. As far as we know, there have only been two other cases of a paediatric extra-axial medulloblastoma in the cerebellar hemisphere reported in the literature. After initial medical stabilisation and insertion of an external ventricular drain, further imaging was obtained. MRI demonstrated a heterogeneously enhancing mass with diffusion restriction, a dural tail and a CSF cleft in the left lateral cerebellar hemisphere. A far lateral craniotomy was performed, with the tumour being encountered immediately on dural opening and a defined cleavage plane seen between the tumour and the cerebellar hemisphere. Gross total resection was achieved, and after a short recovery period, adjuvant craniospinal irradiation plus chemotherapy is started. This case illustrates an exceptionally rare location of a relatively common paediatric malignant brain tumour.</p>","PeriodicalId":9080,"journal":{"name":"BMJ Case Reports","volume":"18 4","pages":""},"PeriodicalIF":0.6000,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Paediatric extra-axial medulloblastoma presenting with obstructive hydrocephalus and tonsillar herniation: clinical presentation and treatment challenges.\",\"authors\":\"Linyi Zhu, Amer Helal, Xenia Doorenbosch\",\"doi\":\"10.1136/bcr-2024-263710\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Medulloblastoma is the most common paediatric tumour presenting in the posterior fossa. Typically, they are found midline and intra-axially located. Medulloblastomas presenting in an extra-axial location, however, are rare, and of those, they are usually found in the cerebellopontine angle. We present a case of an extra-axial medulloblastoma projecting from the lateral cerebellar hemisphere. As far as we know, there have only been two other cases of a paediatric extra-axial medulloblastoma in the cerebellar hemisphere reported in the literature. After initial medical stabilisation and insertion of an external ventricular drain, further imaging was obtained. MRI demonstrated a heterogeneously enhancing mass with diffusion restriction, a dural tail and a CSF cleft in the left lateral cerebellar hemisphere. A far lateral craniotomy was performed, with the tumour being encountered immediately on dural opening and a defined cleavage plane seen between the tumour and the cerebellar hemisphere. Gross total resection was achieved, and after a short recovery period, adjuvant craniospinal irradiation plus chemotherapy is started. This case illustrates an exceptionally rare location of a relatively common paediatric malignant brain tumour.</p>\",\"PeriodicalId\":9080,\"journal\":{\"name\":\"BMJ Case Reports\",\"volume\":\"18 4\",\"pages\":\"\"},\"PeriodicalIF\":0.6000,\"publicationDate\":\"2025-04-08\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"BMJ Case Reports\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1136/bcr-2024-263710\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"BMJ Case Reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1136/bcr-2024-263710","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
Paediatric extra-axial medulloblastoma presenting with obstructive hydrocephalus and tonsillar herniation: clinical presentation and treatment challenges.
Medulloblastoma is the most common paediatric tumour presenting in the posterior fossa. Typically, they are found midline and intra-axially located. Medulloblastomas presenting in an extra-axial location, however, are rare, and of those, they are usually found in the cerebellopontine angle. We present a case of an extra-axial medulloblastoma projecting from the lateral cerebellar hemisphere. As far as we know, there have only been two other cases of a paediatric extra-axial medulloblastoma in the cerebellar hemisphere reported in the literature. After initial medical stabilisation and insertion of an external ventricular drain, further imaging was obtained. MRI demonstrated a heterogeneously enhancing mass with diffusion restriction, a dural tail and a CSF cleft in the left lateral cerebellar hemisphere. A far lateral craniotomy was performed, with the tumour being encountered immediately on dural opening and a defined cleavage plane seen between the tumour and the cerebellar hemisphere. Gross total resection was achieved, and after a short recovery period, adjuvant craniospinal irradiation plus chemotherapy is started. This case illustrates an exceptionally rare location of a relatively common paediatric malignant brain tumour.
期刊介绍:
BMJ Case Reports is an important educational resource offering a high volume of cases in all disciplines so that healthcare professionals, researchers and others can easily find clinically important information on common and rare conditions. All articles are peer reviewed and copy edited before publication. BMJ Case Reports is not an edition or supplement of the BMJ.