罕见部位的常见肿瘤:小脑胶质母细胞瘤单中心病例系列。

IF 1 4区 医学 Q4 CLINICAL NEUROLOGY
Ashwin Kumaria, Alex J Leggate, Graham R Dow, Harshal A Ingale, Iain J A Robertson, Paul O Byrne, Surajit Basu, Donald C Macarthur, Stuart J Smith
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引用次数: 0

摘要

虽然胶质母细胞瘤是成人中最常见的原发性脑肿瘤,但它位于小脑的情况却极为罕见。我们介绍了 13 例(3 例女性,10 例男性;发病时的中位年龄为 56 岁 [年龄范围为 21-77 岁])经手术治疗、组织学确诊的原发性小脑胶质母细胞瘤(cGB)病例,这些病例的病程长达 17 年(2005-2022 年)。鉴于小脑胶质母细胞瘤的罕见性,术前放射学诊断具有挑战性,尽管所有病例的磁共振成像均显示环形强化。手术治疗包括 11 例后窝颅骨切除术和清扫术,以及 2 例毛刺孔活检术。4例病例需要进行脑脊液转移。检测中未发现 IDH 或 ATRX 基因突变的证据。确诊后的存活期为 1 到 22 个月(平均 10.9 个月)。我们还试图了解为什么胶质母细胞瘤在这一部位罕见,并讨论其潜在原因。我们假设,小脑与胚芽区的解剖学距离越来越远,当地内源性神经干细胞活性降低(与胶质母细胞瘤有关),这可能是胶质母细胞瘤在小脑罕见的原因。这是迄今为止英国规模最大的小脑胶质母细胞瘤系列研究,因此我们试图为有限的小脑胶质母细胞瘤文献增添新的内容。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A common tumour in a rare location: a single centre case series of cerebellar glioblastoma.

Although glioblastoma is the commonest primary brain tumour in adults, its location in the cerebellum is extremely rare. We present thirteen cases (3 female, 10 male; median age at presentation 56 [age range 21-77]) of surgically managed, histologically confirmed, primary cerebellar glioblastoma (cGB) over a 17 year period (2005-2022). Pre-operative radiological diagnosis was challenging given cGB rarity, although MRI demonstrated ring enhancement in all cases. Surgical management included posterior fossa craniectomy and debulking in 11 cases and burr hole biopsy in two. CSF diversion was necessary in four cases. No evidence of IDH or ATRX gene mutations was found when tested. Survival ranged from 1 to 22 months after diagnosis (mean 10.9 months). We also seek to understand why glioblastoma is rare in this location and discuss potential reasons for this. We hypothesise that increasing anatomical distance from germinal regions and decreased local endogenous neural stem cell activity (which has been associated with glioblastoma) may explain why glioblastoma is rare in the cerebellum. We hereby seek to add to the limited literature on cGB as this is the largest UK cGB series to date.

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来源期刊
British Journal of Neurosurgery
British Journal of Neurosurgery 医学-临床神经学
CiteScore
2.30
自引率
9.10%
发文量
139
审稿时长
3-8 weeks
期刊介绍: The British Journal of Neurosurgery is a leading international forum for debate in the field of neurosurgery, publishing original peer-reviewed articles of the highest quality, along with comment and correspondence on all topics of current interest to neurosurgeons worldwide. Coverage includes all aspects of case assessment and surgical practice, as well as wide-ranging research, with an emphasis on clinical rather than experimental material. Special emphasis is placed on postgraduate education with review articles on basic neurosciences and on the theory behind advances in techniques, investigation and clinical management. All papers are submitted to rigorous and independent peer-review, ensuring the journal’s wide citation and its appearance in the major abstracting and indexing services.
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