Department of Neurosurgery, Xuanwu Hospital, China International Neuroscience Institute (China -INI), Capital Medical University, Beijing, China.
Pediatr Neurosurg. 2022;57(6):422-433. doi: 10.1159/000526541. Epub 2022 Aug 16.
Pediatric meningiomas (PMs) are rare tumors; they differ from their adult counterparts by their atypicality of location, higher rates of malignant change, male preponderance, recurrence, and sometimes, their association with neurofibromatosis. This case series analyzes the clinical behavior, pathological presentation, location, and its association with neurofibromatosis type 2 (NF2).
This case series consists of pediatric patients between the ages of 4 and 16 years who were hospitalized in the neurosurgical department of our hospital from 2012 to 2021 with different neurological symptoms and a literature review using the PubMed/MEDLINE database.
Sixty percent of the patients were males, while 40% were females. The most common neurological manifestations were signs of increased intracranial pressure. NF2 was absent in all patients. The predominant histopathology subtypes are atypical and WHO grade II, representing 30% and 40%, respectively.
This study supports the relationship between NF2 and pediatric cerebral meningioma but at a lower concomitant rate from 0 to 13%, taking into consideration our original data and the literature review, contrasting some reported cases, which suggest rates as high as 33%, 50%, and 100% in a very small number of patients. Gross total resection without postoperative radiation therapy for nonmalignant and non-NF2-associated PM proved to be a sufficient and a good treatment option.
小儿脑膜瘤（PMs）是罕见的肿瘤；它们与成人脑膜瘤不同，其位置不典型，恶性变化率较高，男性居多，易复发，有时与神经纤维瘤病 2 型（NF2）相关。本病例系列分析了临床行为、病理表现、位置及其与 NF2 的关系。
本病例系列包括 2012 年至 2021 年期间在我院神经外科住院的 4 至 16 岁小儿患者，他们具有不同的神经症状，并使用 PubMed/MEDLINE 数据库进行文献复习。
60%的患者为男性，40%为女性。最常见的神经表现为颅内压增高的体征。所有患者均无 NF2。主要组织病理学亚型为非典型和 WHO 分级 II，分别占 30%和 40%。
本研究支持 NF2 与小儿脑脑膜瘤之间的关系，但同时发病率较低，从 0 到 13%，考虑到我们的原始数据和文献复习，与一些报道的病例形成对比，这些病例报告在极少数患者中发病率高达 33%、50%和 100%。对于非恶性和非 NF2 相关的 PM，行无术后放疗的大体全切除被证明是一种充分且良好的治疗选择。
