Department of Neurosurgery, The First Hospital of Jilin University, Changchun, China.
Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China.
Childs Nerv Syst. 2021 Aug;37(8):2661-2665. doi: 10.1007/s00381-020-04975-y. Epub 2020 Nov 20.
Clear cell meningiomas (CCMs) account for only approximately 0.2% of all meningiomas and are classified as grade II tumours by the World Health Organisation. Most of the previously described intracranial CCMs are single tumour entities. Multiple intracranial CCMs are extremely rare, and due to their low incidence, information on multiple CCM remains limited; hence, researchers and practitioners also have a limited understanding of its aetiology, clinical manifestations, and treatment options. Herein, we report a case of sporadic multiple intracranial CCM in a child that presented with a 3-month history of gait instability and mild dysphagia. Brain magnetic resonance imaging showed one lesion in the right frontoparietal lobe and another giant lesion on the ventral side of the brainstem, extending to the middle fossa. The lesions were completely resected in two stages. Postoperative histopathological examination confirmed CCM. The patient was followed up for 2 years without recurrence. To the best of our knowledge this is the first reported case of paediatric sporadic multiple intracranial CCM. In addition, we reviewed the relevant literature to discuss the pathogenesis, imaging findings, and treatment methods of this rare disease.
透明细胞脑膜瘤（CCMs）仅占所有脑膜瘤的约 0.2%，并被世界卫生组织归类为二级肿瘤。以前描述的大多数颅内 CCM 都是单一肿瘤实体。颅内多发 CCM 极为罕见，由于其发病率低，有关多发 CCM 的信息仍然有限；因此，研究人员和从业者对其病因、临床表现和治疗选择的了解也很有限。在此，我们报告一例儿童散发性多发颅内 CCM 病例，该患儿表现为步态不稳和轻度吞咽困难 3 个月。脑部磁共振成像显示右额顶叶有一个病变，另一个巨大病变位于脑干腹侧，延伸至中颅窝。这些病变分两期完全切除。术后组织病理学检查证实为 CCM。患者随访 2 年无复发。据我们所知，这是首例报道的儿童散发性多发颅内 CCM。此外，我们回顾了相关文献，讨论了这种罕见疾病的发病机制、影像学表现和治疗方法。
