Department of Neurosciences and Reproductive and Odontostomatological Sciences, Neurosurgical Clinic, School of Medicine, University "Federico II", Naples, Italy.
Department of Advanced Biomorphological Sciences, Section of Pathology, School of Medicine, University "Federico II", Naples, Italy.
Neurosurg Rev. 2022 Feb;45(1):151-166. doi: 10.1007/s10143-021-01585-3. Epub 2021 Jun 23.
Malignant intraventricular meningiomas (IVMs) are very rare with only a few reported cases. A midline search up to December 2020 selected 40 articles for a total of 65 patients. The inclusion criteria were series and case reports in English language, as well as papers written in other languages, but with abstracts written in English. Malignant IVMs at the first diagnosis (group A, 50 patients) and those with anaplastic transformation from previous WHO grades I and II tumors (group B, 15 patients) were separately analyzed. The unique personal case among 1285 meningiomas (0.078%) is also added. Malignant IVMs mainly occur in women (61%) with a median age of 45 years and are mainly located in the lateral ventricle (93%) and trigonal region (74%), with no cases in the fourth ventricle. Irregular borders (80%), heterogeneous enhancement (83%), and perilesional edema (76%) are the most frequent radiological findings. The histology was mainly pure anaplastic (85%), whereas papillary (7%), rhabdoid (5%), and mixed forms (3%) are very rare. The CSF spread was found in 60% of the cases. The prognosis is very dismal, with an overall median survival of 17.5 months after surgery for the anaplastic forms. Malignant IVMs at initial diagnosis (group A) show better overall survival (25 months) than those occurring from anaplastic transformation of lower grade tumors (group B) (10.1 months).
恶性脑室内脑膜瘤（IVM）非常罕见，仅有少数报道。截至 2020 年 12 月，通过中线搜索，共选择了 40 篇文章，总计 65 例患者。纳入标准为英文系列病例报告和病例报告，以及其他语言撰写但英文摘要的论文。首次诊断为恶性 IVM（A 组，50 例）和从以前的 I 级和 II 级肿瘤发生间变的患者（B 组，15 例）分别进行了分析。还增加了 1285 例脑膜瘤中唯一的 1 例（0.078%）个人病例。恶性 IVM 主要发生在女性（61%），中位年龄为 45 岁，主要位于侧脑室（93%）和三角区（74%），第四脑室无病例。边界不规则（80%）、不均匀强化（83%）和瘤周水肿（76%）是最常见的影像学表现。组织学主要为纯间变（85%），而乳头状（7%）、横纹肌样（5%）和混合形式（3%）非常罕见。在 60%的病例中发现了 CSF 播散。预后非常差，间变形式手术后总体中位生存期为 17.5 个月。初诊时的恶性 IVM（A 组）的总体生存率（25 个月）优于由低级肿瘤间变而来的恶性 IVM（B 组）（10.1 个月）。
