Dietrich P Y, Aapro M S, Rieder A, Pizzolato G P
Department of Medicine, University Hospital, Geneva, Switzerland.
J Neurooncol. 1993 Mar;15(3):275-83. doi: 10.1007/BF01050075.
Cancerous 'chronic meningitis' may be related to subarachnoid space involvement by solid tumors, hematologic malignancies or rarely intraparenchymatous gliomas. Primary leptomeningeal gliomatosis is a rare condition that is attributed to malignant transformation of heterotopic neuroglial tissue. We discuss the clinical and biological features of a patient who died with the diffuse form of primary leptomeningeal gliomatosis (PDLG). A literature search shows that a one to two months long non-specific prodromal phase followed by a fluctuating neurologic downhill course is suggestive of this disease. Cerebro-spinal fluid (CSF) cytology has been diagnostic in only 1 of 8 reported cases. Recent technical progress, including the use of GFAP (glial fibrillary acidic protein) directed antibody, may enhance the sensitivity of CSF cytologies. Diagnosis may require repeated cerebral biopsies, because the hemispheric lesions are often separated by normal tissue. PDLG must be added to the large differential diagnosis of 'chronic meningitis'.
癌性“慢性脑膜炎”可能与实体瘤、血液系统恶性肿瘤或罕见的脑实质内胶质瘤侵犯蛛网膜下腔有关。原发性软脑膜胶质瘤病是一种罕见疾病，归因于异位神经胶质组织的恶性转化。我们讨论了一名死于弥漫性原发性软脑膜胶质瘤病（PDLG）患者的临床和生物学特征。文献检索表明，长达一到两个月的非特异性前驱期，随后是神经系统症状波动的恶化病程，提示了这种疾病。脑脊液（CSF）细胞学检查在8例报告病例中仅1例具有诊断价值。包括使用胶质纤维酸性蛋白（GFAP）导向抗体在内的近期技术进展，可能会提高脑脊液细胞学检查的敏感性。诊断可能需要重复进行脑活检，因为半球病变常被正常组织分隔。在“慢性脑膜炎”的众多鉴别诊断中必须加入PDLG。
