Lepreux Sébastien, Sagnier Sharmilla, Perez Jean-Thomas, Léger François, Sibon Igor, Vital Anne
Clin Neuropathol. 2017 Sep/Oct;36(5):222-226. doi: 10.5414/NP301018.
Primary diffuse leptomeningeal gliomatosis (PDLG) is characterized by diffuse infiltration of the leptomeningeal space by neoplastic glial cells without evidence of intra-parenchymatous primary tumor. We report a case of PDLG in a 68-year-old man, who died 1 month after onset of symptoms. The diagnosis was made on autopsy data. We discuss the particularities of this entity, which is not registered in the WHO classification of tumors of the central nervous system (2016). In case of an unexplained inflammatory meningeal process and in the presence of atypical cells in the cerebrospinal fluid, PDLG needs to be considered. This diagnosis of PDLG has to be confirmed by meningeal imaging-guided biopsy, which must be repeated if necessary. .
原发性弥漫性软脑膜胶质瘤病（PDLG）的特征是肿瘤性胶质细胞弥漫浸润软脑膜间隙，而无脑实质内原发性肿瘤的证据。我们报告一例68岁男性的PDLG病例，该患者在症状出现后1个月死亡。诊断基于尸检数据。我们讨论了这一实体的特殊性，它未被列入世界卫生组织中枢神经系统肿瘤分类（2016年）。在出现无法解释的炎性脑膜病变且脑脊液中存在非典型细胞的情况下，需要考虑PDLG。PDLG的诊断必须通过脑膜成像引导下的活检来证实，如有必要，必须重复进行。
