Astrocytomas IDH-mutant of posterior cranial fossa, clinical presentation, imaging features and onco-functional balance in surgical management.

IF 2.5 3区 医学 Q2 CLINICAL NEUROLOGY
Matteo De Simone, Anis Choucha, Carlotta Ranalli, Giuseppina Pecoraro, Romain Appay, Oliver L Chinot, Henry Dufour, Giorgio Iaconetta
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Abstract

IDH-mutant astrocytomas (AIDHmut) in the posterior cranial fossa (PCF) are rare and present substantial diagnostic and therapeutic challenges due to their location. We analyzed patients with PCF AIDHmut from our institutions, treated between December 2021 and September 2024. Additionally, we conducted a systematic literature review (from January 2021 to September 2024) using PubMed, Ovid MEDLINE, and Ovid EMBASE to identify cases of PCF AIDHmut. We identified a total of 19 cases, including one institutional case. Most patients were young adults, with a male predominance (15 males, 4 females). Tumors primarily originated from the brainstem (94.7%), with only one case involving the cerebellum. Clinical presentations frequently included cranial nerve deficits, with diplopia being the most common symptom (47.4%). Adjuvant radiotherapy (IMRT, DT 54 Gy/27 fractions, 78.9%) and chemotherapy (temozolomide, 68.4%) formed the mainstays of treatment. Tumor grading revealed 63.2% (12/19) were WHO grade 2, 21% (4/19) were WHO grade 3, and 15.8% (3/19) were grade 4. The mean follow-up period was 45 months. PCF AIDHmut are rare but pose significant treatment challenges due to their location and infiltrative nature. Multimodal treatment-comprising surgery, radiotherapy, and chemotherapy-is essential for achieving long-term disease control. Subtotal resection followed by adjuvant therapies provides a favorable balance between tumor control and functional preservation.

后颅窝星形细胞瘤idh突变,临床表现,影像学特征和手术治疗中的单功能平衡。
后颅窝(PCF)的idh突变型星形细胞瘤(AIDHmut)是罕见的,由于其位置的原因,存在大量的诊断和治疗挑战。我们分析了来自我们机构的PCF AIDHmut患者,这些患者在2021年12月至2024年9月期间接受治疗。此外,我们使用PubMed、Ovid MEDLINE和Ovid EMBASE进行了系统的文献综述(从2021年1月到2024年9月),以确定PCF AIDHmut病例。我们共确定了19例,其中包括1例机构病例。患者多为青壮年,以男性为主(男性15例,女性4例)。肿瘤主要起源于脑干(94.7%),仅有一例累及小脑。临床表现通常包括颅神经缺损,复视是最常见的症状(47.4%)。辅助放疗(IMRT, DT 54 Gy/27分次,78.9%)和化疗(替莫唑胺,68.4%)是治疗的支柱。肿瘤分级63.2%(12/19)为WHO 2级,21%(4/19)为WHO 3级,15.8%(3/19)为WHO 4级。平均随访时间为45个月。PCF AIDHmut是罕见的,但由于其位置和浸润性,给治疗带来了重大挑战。多模式治疗——包括手术、放疗和化疗——对于实现疾病的长期控制至关重要。次全切除后辅助治疗在肿瘤控制和功能保存之间提供了良好的平衡。
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来源期刊
Neurosurgical Review
Neurosurgical Review 医学-临床神经学
CiteScore
5.60
自引率
7.10%
发文量
191
审稿时长
6-12 weeks
期刊介绍: The goal of Neurosurgical Review is to provide a forum for comprehensive reviews on current issues in neurosurgery. Each issue contains up to three reviews, reflecting all important aspects of one topic (a disease or a surgical approach). Comments by a panel of experts within the same issue complete the topic. By providing comprehensive coverage of one topic per issue, Neurosurgical Review combines the topicality of professional journals with the indepth treatment of a monograph. Original papers of high quality are also welcome.
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