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Primary diffuse leptomeningeal gliomatosis: do we miss the diagnosis?
Abstract:
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. .
Insights
Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare condition where glial tumors spread diffusely in the meninges. This case highlights diagnostic challenges and emphasizes considering PDLG in unexplained meningeal inflammation.
Area of Science:
- Neurology
- Oncology
- Pathology
Background:
- Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare neoplastic condition.
- It involves diffuse leptomeningeal infiltration by glial cells without an apparent primary parenchymal tumor.
- PDLG is not currently classified in the WHO classification of CNS tumors (2016).
