Department of Neurosurgery, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan.
Department of Neurosurgery, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan.
World Neurosurg. 2020 Jul;139:535-547. doi: 10.1016/j.wneu.2020.04.152. Epub 2020 May 1.
Pediatric meningiomas account for less than 2% of pediatric brain tumors. Pediatric clear cell meningiomas (CCMs) occurring in the posterior fossa are particularly rare. Therapeutic strategies differ among the previous pediatric CCM case reports. Therefore, to clarify the clinical features of pediatric CCMs, we report a rare case of dumbbell-type pediatric CCM and a corresponding literature review.
A 7-year-old boy with complaints of headache, left facial palsy, dysarthria, and left-sided ataxic gait was emergently admitted to our hospital. His consciousness level was slight stupor, with Glasgow Coma Scale score 3-5-6, and he showed left ptosis, dysarthria, and ataxias of the left trunk and extremities. Magnetic resonance imaging (MRI) scan showed acute obstructive hydrocephalus because of the tumor's compression of the brainstem. The dumbbell-shaped tumor extended from the lateral wall of the cavernous sinus, through the left Meckel's cave, to the cerebellopontine angle. Physical examination and perioperative MRI scan showed no evidence regarding neurofibromatosis type I or II. The tumor was removed in a 2-staged operation. Postoperative proton therapy was done to treat some residual tumors. One year after postoperative proton therapy, there is no recurrence, and apart from left corneal and facial hypesthesia, he is healthy.
We reported a rare case of pediatric skull base-type CCM with huge extension originating from the anteromedial wall of Meckel's cave firmly adhered to the cavernous sinus wall to the posterior fossa that was successfully treated with surgery and postoperative proton therapy. CCM has a high recurrence rate; therefore, careful prolonged follow-up is needed.
儿童脑膜瘤占儿童脑肿瘤的比例不到 2%。发生在后颅窝的儿童透明细胞脑膜瘤（CCM）尤其罕见。以往的儿童 CCM 病例报告中，治疗策略有所不同。因此，为了阐明儿童 CCM 的临床特征，我们报告了一例罕见的哑铃型儿童 CCM 病例，并进行了相应的文献复习。
一名 7 岁男孩因头痛、左侧面瘫、构音障碍和左侧共济失调步态而紧急入院。他的意识水平轻度昏迷，格拉斯哥昏迷量表评分为 3-5-6，表现为左侧上睑下垂、构音障碍和左侧躯干及四肢共济失调。磁共振成像（MRI）扫描显示由于肿瘤压迫脑干导致急性梗阻性脑积水。哑铃形肿瘤从海绵窦的外侧壁延伸，通过左侧 Meckel 氏腔，到达桥小脑角。体格检查和围手术期 MRI 扫描均未发现神经纤维瘤病 I 型或 II 型的证据。肿瘤分两期切除。术后行质子治疗以治疗部分残留肿瘤。术后质子治疗 1 年后，无复发，除左侧角膜和面部感觉减退外，患者健康状况良好。
我们报告了一例罕见的儿童颅底型 CCM 病例，该肿瘤起源于 Meckel 氏腔的前内侧壁，牢固地附着于海绵窦壁，向后方延伸至后颅窝，通过手术和术后质子治疗成功治疗。CCM 复发率高，因此需要仔细、长期的随访。
