Gonda Tomomi, Wakabayashi Kenichi, Haraguchi Kenichi, Arai Yoshifumi, Oyama Hirofumi
Department of Neurosurgery, Nagoya University Graduate School of Medicine.
Department of Neurosurgery, Toyohashi Municipal Hospital.
Rinsho Shinkeigaku. 2021 Dec 22;61(12):862-868. doi: 10.5692/clinicalneurol.cn-001661. Epub 2021 Nov 18.
A 35-year-old man was admitted to our department for loss of consciousness. CT and MRI revealed diffuse enhancement of the subarachnoid space surrounding the brainstem and the cerebellar sulci, without any parenchymal lesions in the brain or the spinal cord. Furthermore, gadolinium-enhanced MRI revealed a nodular lesion with heterogeneous enhancement in the right prepontine cistern, at the site from which a biopsy was obtained via right lateral suboccipital craniotomy on the day following admission. Histopathological examination of the resected specimen revealed glioblastoma multiforme. Based on the radiological and histopathological findings, the patient was diagnosed with primary leptomeningeal gliomatosis (PLG). The patient received temozolomide chemotherapy with concurrent radiotherapy and showed radiological remission, 12 months after diagnosis. However, he developed local recurrence 6 months later and died 23 months after diagnosis. Autopsy findings showed tumor cell infiltration of the leptomeninges, as well as the brain and spinal parenchyma. PLG should be considered in the differential diagnosis in patients with diffuse leptomeningeal enhancement even without parenchymal lesions on radiological imaging. A surgical biopsy is recommended for prompt and accurate diagnosis in such cases.
一名35岁男性因意识丧失入住我科。CT和MRI显示脑干及小脑沟周围蛛网膜下腔弥漫性强化,脑和脊髓实质无任何病变。此外,钆增强MRI显示右侧脑桥前池有一个结节状病变,强化不均匀,入院次日经右侧枕下开颅手术从此处获取活检标本。切除标本的组织病理学检查显示为多形性胶质母细胞瘤。根据影像学和组织病理学结果,该患者被诊断为原发性软脑膜胶质瘤病(PLG)。患者接受了替莫唑胺化疗并同步放疗,诊断后12个月显示影像学缓解。然而,6个月后出现局部复发,诊断后23个月死亡。尸检结果显示软脑膜、脑和脊髓实质均有肿瘤细胞浸润。即使影像学检查无实质病变,对于弥漫性软脑膜强化的患者,鉴别诊断时也应考虑PLG。对于此类病例,建议进行手术活检以迅速准确诊断。