背景:小儿脑膜瘤(PM)是罕见的肿瘤;它们与成年人的位置不典型不同,恶性变率较高,男性优势,复发,有时,它们与神经纤维瘤病有关。这个案例系列分析了临床行为,病理表现,location,及其与2型神经纤维瘤病(NF2)的关系。
方法:本病例系列包括2012年至2021年在我院神经外科住院的年龄在4至16岁之间的儿科患者,并使用PubMed/MEDLINE数据库进行文献综述。
结果:60%的患者是男性,而40%是女性。最常见的神经系统表现是颅内压升高的迹象。所有患者均不存在NF2。主要的组织病理学亚型是非典型和WHOII级,占30%和40%,分别。
结论:这项研究支持NF2与小儿脑膜瘤之间的关系,但伴随率从0%到13%较低,考虑到我们的原始数据和文献综述,对比一些报道的病例,这表明利率高达33%,50%,在极少数患者中100%。对于非恶性和非NF2相关的PM,不进行术后放射治疗的总切除被证明是足够且良好的治疗选择。
BACKGROUND: 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).
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.