Narayan Vinayak, Savardekar Amey, Mohammed Nasser, Patra Devi Prasad, Georgescu Maria-Magdalena, Nanda Anil
Department of Neurosurgery, Louisiana State University Health Sciences Center, Shreveport, Louisiana, USA.
Division of Neuropathology, Department of Pathology, Louisiana State University Health Sciences Center, Shreveport, Louisiana, USA.
World Neurosurg. 2018 Aug;116:163-168. doi: 10.1016/j.wneu.2018.04.080. Epub 2018 Apr 22.
Primary focal intracranial leptomeningeal glioma (PFILG) is considered a rare solitary glial tumor arising from the leptomeninges without brain attachment or evidence of primary neoplasm elsewhere within the neuraxis. We report a case of PFILG in a left parietal location and provide a concise review on its clinicoradiologic, histopathologic, and management characteristics.
A 75-year-old woman presented with focal motor seizures involving the right upper limb with associated occasional headache. Magnetic resonance imaging (MRI) of the brain revealed a small focal lesion attached to the left parietal dura with underlying brain parenchymal signal changes, and contrast-enhanced images showed heterogeneous enhancement of the tumor and meninges. The repeated MRI brain images within a month of primary imaging revealed significant progression of the size of lesion along with invasion of underlying parietal lobe parenchyma. The patient underwent gross total resection of the lesion, and the histopathologic diagnosis was glioblastoma multiforme (GBM), World Health Organization grade 4, isocitrate dehydrogenase wild type. The patient recovered well from surgery without deficits; however, she refused adjuvant treatment. MRI of the brain repeated 3 months after surgery revealed significant progression of the GBM with mass effect. Although adjuvant treatment was then started, she could not tolerate it and died 4 months after surgery.
The possibility of high-grade PFILG should be considered in any aggressive extra-axial focal lesion. The definitive diagnosis can be made after histologic examination. Although surgery followed by adjuvant treatment is considered the mainstay of treatment, the overall prognostic outcome of high-grade PFILG is dismal.
原发性局灶性颅内软脑膜胶质瘤（PFILG）被认为是一种罕见的孤立性胶质肿瘤，起源于软脑膜，与脑无附着关系，且在神经轴内其他部位无原发性肿瘤证据。我们报告一例位于左侧顶叶的PFILG病例，并对其临床放射学、组织病理学及治疗特征进行简要综述。
一名75岁女性，出现累及右上肢的局灶性运动性癫痫发作，并伴有偶尔头痛。脑部磁共振成像（MRI）显示一个小的局灶性病变附着于左侧顶叶硬脑膜，其下方脑实质信号改变，增强图像显示肿瘤和脑膜不均匀强化。初次成像后一个月内重复的脑部MRI图像显示病变大小显著进展，同时累及下方顶叶实质。患者接受了病变的全切手术，组织病理学诊断为多形性胶质母细胞瘤（GBM），世界卫生组织4级，异柠檬酸脱氢酶野生型。患者术后恢复良好，无功能缺损；然而，她拒绝辅助治疗。术后3个月重复脑部MRI显示GBM显著进展并出现占位效应。尽管随后开始了辅助治疗，但她无法耐受，术后4个月死亡。
对于任何侵袭性轴外局灶性病变，均应考虑高级别PFILG的可能性。组织学检查后可做出明确诊断。尽管手术加辅助治疗被认为是主要治疗方法，但高级别PFILG的总体预后较差。
