Objective:This systematic review consolidates the literature on primary extradural meningiomas (PEMs), a rare subset of meningiomas. We describe the clinical features, management strategies used, and treatment outcomes for published cases.Methods:A systematic review was conducted using PRISMA guidelines across multiple databases to 29 July 2024. Inclusion criteria were adult patients with primarily extradural meningioma and where individual patient clinical data were provided.Results:Of 216 studies that met the initial search criteria, 41 satisfied the final inclusion criteria. These 41 studies included 82 patients with 84 total PEMs. The cohort was balanced between sexes with a median age of 46 (range 18–82). Frequent symptoms at initial presentation included pain/headache (46%), weakness (44%), paresthesias (24%), and a palpable superficial mass (23%). The median duration of symptoms to diagnosis was 11 months (range 0.75–120). Surgical resection was the primary treatment approach, achieving a gross total resection in 67% of cases. The majority of lesions were classified as WHO grade 1 (87%). A recurrence was identified during the published follow-up in 11% of cases and a higher WHO grade was expectedly associated with a greater risk of recurrence. The described practice was to use adjuvant radiotherapy in recurrent and high-grade cases. Most cranial lesions were located in the frontal bone, while most spinal lesions affected the cervical spine. Post-treatment symptom improvement or resolution was described in almost all patients at the last follow-up.Conclusions:In comparison to intradural meningiomas, PEMs largely follow a more indolent course with a longer duration of symptoms prior to diagnosis, more benign symptoms, a higher proportion of grade 1 tumors, and favorable outcomes; however, there is a small subset of PEMs with extension outside the cranium and spine that require specific considerations for management.
目的：本系统综述整合了关于原发性硬膜外脑膜瘤（PEMs）的文献，这是一种罕见的脑膜瘤亚型。我们描述了已发表病例的临床特征、治疗策略及治疗结果。 方法：遵循PRISMA指南，对截至2024年7月29日的多个数据库进行了系统综述。纳入标准为成年原发性硬膜外脑膜瘤患者，且提供了个体患者临床数据。 结果：在符合初步检索标准的216项研究中，41项符合最终纳入标准。这41项研究共包括82名患者，总计84个PEMs。队列性别分布均衡，中位年龄为46岁（范围18-82岁）。初诊时常见症状包括疼痛/头痛（46%）、无力（44%）、感觉异常（24%）以及可触及的浅表肿块（23%）。从症状出现到诊断的中位时间为11个月（范围0.75-120个月）。手术切除是主要治疗方法，67%的病例实现了大体全切除。大多数病变被归类为WHO 1级（87%）。在已发表的随访中，11%的病例出现复发，且更高的WHO分级与更高的复发风险相关。文献中描述的做法是在复发和高级别病例中使用辅助放疗。大多数颅骨病变位于额骨，而大多数脊柱病变影响颈椎。在末次随访时，几乎所有患者均描述了治疗后症状改善或消失。 结论：与硬膜内脑膜瘤相比，PEMs通常病程更为惰性，诊断前症状持续时间更长，症状更良性，1级肿瘤比例更高，且预后良好；然而，存在一小部分PEMs向颅骨和脊柱外延伸，其治疗需要特殊考虑。
肿瘤(癌症)患者之家