Grilli Fulvio, Smaili Hamzah Youssef, Bianchi Federico, Frassanito Paolo, Tamburrini Gianpiero, Massimi Luca
Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Rome, Italy.
Hospital Infantil Sabarà - Sao Paulo, São Paulo, Brazil.
Childs Nerv Syst. 2024 Dec 11;41(1):43. doi: 10.1007/s00381-024-06708-x.
Meningiomas are rare tumors in children compared with adults. Their main peculiarities are the frequent convexity or intraventricular location, the common association with neurofibromatosis-2 (NF-2) and the relatively high rate of aggressive and/or hyperostotic variants. Hyperostosis may complicate the surgical management. The goal of this paper is to provide an update on the main characteristics and the management of hyperostotic meningiomas.
A 7-year-old girl was admitted to our department because of a long-lasting history of left frontal skull bulging. Neuroimaging examinations revealed a huge hyperostotic meningioma with bony invasion and infiltration of the superior sagittal sinus. Genetic tests were positive for NF-2. The tumor (atypical meningioma) was gross totally resected. During the same operation, a custom-made cranioplasty was realized with the help of a frame-based craniectomy. The patient is asymptomatic and under oncological follow-up.
Although generally rare, hyperostotic meningiomas are relatively common in children and clinically demanding because of their large size, the possible aggressive behavior and the need of a cranioplasty as additional surgical step. Therefore, a careful preoperative surgical planning is required, taking into account that the extent of surgical resection is an important prognostic factor. The long-term outcome is good.
与成人相比，脑膜瘤在儿童中较为罕见。其主要特点是常位于凸面或脑室内，常与神经纤维瘤病2型（NF-2）相关，且侵袭性和/或骨质增生性变异的发生率相对较高。骨质增生可能会使手术治疗复杂化。本文的目的是提供有关骨质增生性脑膜瘤的主要特征及治疗的最新信息。
一名7岁女孩因长期存在左额颅骨隆起病史入住我科。神经影像学检查显示一个巨大的骨质增生性脑膜瘤，伴有骨质侵犯及上矢状窦浸润。基因检测NF-2呈阳性。肿瘤（非典型脑膜瘤）行全切除。在同一手术过程中，借助基于框架的颅骨切除术进行了定制的颅骨成形术。患者无症状，正在接受肿瘤学随访。
尽管骨质增生性脑膜瘤总体上较为罕见，但在儿童中相对常见，且由于其体积大、可能具有侵袭性行为以及需要进行颅骨成形术作为额外的手术步骤，在临床上颇具挑战性。因此，需要进行仔细的术前手术规划，同时要考虑到手术切除范围是一个重要的预后因素。长期预后良好。
