Adult-onset intramedullary teratomas: systematic review with outcome analysis.

IF 2 4区 医学 Q3 CLINICAL NEUROLOGY Acta neurologica Belgica Pub Date : 2024-10-22 DOI:10.1007/s13760-024-02667-x
Berkay Paker, Önder Ertem, Mehmetzeki Yıldız, Deniz Konya
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Abstract

Background and objective: Intramedullary mature teratomas in adults are rare tumors that pose significant diagnostic and therapeutic challenges due to their diverse tissue composition and intramedullary location. This review, which includes an illustrative case, aims to provide a comprehensive overview of the clinical presentation, diagnosis, treatment, and outcomes of adult intramedullary mature teratomas through a systematic review of 89 cases.

Methods: A systematic review was conducted following PRISMA guidelines, using keyword combinations in PubMed, MEDLINE, and Web of Science databases until June 2024. Inclusion criteria were adult patients (> 18 years) with histopathologically confirmed intramedullary mature teratomas. Statistical analyses examined relationships between the extent of resection (EoR), tumor location, and patient outcomes.

Results: Among the 89 cases, the mean age was 39.94 ± 13.52 years, with a male-to-female ratio of 1.39:1. Tumors were most commonly located in the conus region (51.2%). Surgical resection was the primary treatment, with 43 cases undergoing partial resection and 33 gross total resection. No statistically significant differences in outcomes were found between EoR, sex, associated anomalies, or specific outcomes. However, younger patients showed improved outcomes for lower limb weakness and sphincter dysfunction. Recurrence was noted in three cases, all located in the conus.

Conclusion: Intramedullary mature teratomas require a nuanced approach that balances complete resection and neurological preservation. Early diagnosis and individualized surgical planning are crucial for optimizing outcomes. Despite the challenges, effective management is achievable, and ongoing research is essential to refining treatment strategies for this rare tumor.

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成人髓内畸胎瘤:系统回顾与结果分析。
背景和目的:成人髓内成熟畸胎瘤是一种罕见肿瘤,由于其组织成分和髓内位置的多样性,给诊断和治疗带来了巨大挑战。本综述包括一个示例病例,旨在通过对 89 个病例的系统综述,全面概述成人髓内成熟畸胎瘤的临床表现、诊断、治疗和结果:方法:根据 PRISMA 指南,使用 PubMed、MEDLINE 和 Web of Science 数据库中的关键词组合,对截至 2024 年 6 月的文章进行了系统性回顾。纳入标准为经组织病理学证实患有髓内成熟畸胎瘤的成年患者(大于 18 岁)。统计分析研究了切除范围(EoR)、肿瘤位置和患者预后之间的关系:89例患者的平均年龄为(39.94 ± 13.52)岁,男女比例为1.39:1。肿瘤最常位于锥体部位(51.2%)。手术切除是主要治疗方法,其中43例进行了部分切除,33例进行了全部切除。在EoR、性别、相关异常或特定结果之间,未发现统计学上的明显差异。不过,年轻患者在下肢无力和括约肌功能障碍方面的疗效较好。有三个病例出现复发,均位于锥体:结论:髓内成熟畸胎瘤需要一种平衡完全切除和神经保护的微妙方法。早期诊断和个体化手术规划对优化治疗效果至关重要。尽管挑战重重,但有效的治疗是可以实现的,持续的研究对于完善这种罕见肿瘤的治疗策略至关重要。
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来源期刊
Acta neurologica Belgica
Acta neurologica Belgica 医学-临床神经学
CiteScore
4.20
自引率
3.70%
发文量
300
审稿时长
6-12 weeks
期刊介绍: Peer-reviewed and published quarterly, Acta Neurologica Belgicapresents original articles in the clinical and basic neurosciences, and also reports the proceedings and the abstracts of the scientific meetings of the different partner societies. The contents include commentaries, editorials, review articles, case reports, neuro-images of interest, book reviews and letters to the editor. Acta Neurologica Belgica is the official journal of the following national societies: Belgian Neurological Society Belgian Society for Neuroscience Belgian Society of Clinical Neurophysiology Belgian Pediatric Neurology Society Belgian Study Group of Multiple Sclerosis Belgian Stroke Council Belgian Headache Society Belgian Study Group of Neuropathology
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