Primary leptomeningeal gliomatosis: symptoms suggestive of meningitis
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Abstract:
Gliomas that arise primarily in the leptomeninges are rare. Of the 15 reported cases of primary leptomeningeal glioma, only two diffusely involved the leptomeninges. We report the third case of primary leptomeningeal gliomatosis, this in a 55-year-old man who died three months after the onset of symptoms. The clinical manifestations, CSF findings, and the gross appearance of the brain resembled meningitis rather than neoplasm. Results of cytologic examinations of the CSF were normal and the diagnosis was made at necropsy. The diffuse form of primary leptomeningeal glioma has a shorter clinical course and poorer prognosis than the solitary form. Primary leptomeningeal gliomas are believed to arise from heterotopic neuroglial tissue in the leptomeninges.
Insights
Primary leptomeningeal gliomatosis is a rare, aggressive brain tumor. This diffuse form presents like meningitis, leading to delayed diagnosis and poor prognosis, often found only during autopsy.
Area of Science:
- Neuro-oncology
- Neuropathology
Background:
- Primary leptomeningeal gliomas are rare neoplasms originating in the brain's meninges.
- The diffuse form of leptomeningeal glioma is exceptionally uncommon, with only two prior reported cases.
Observation:
- This report details a third case of diffuse primary leptomeningeal gliomatosis in a 55-year-old male.
- Clinical presentation, cerebrospinal fluid (CSF) analysis, and gross brain findings mimicked meningitis.
- Diagnosis was established post-mortem due to normal CSF cytology and non-neoplastic initial clinical suspicion.
Findings:
- The diffuse form of primary leptomeningeal glioma exhibits a rapid clinical course.
- Patients with diffuse leptomeningeal gliomatosis have a significantly poorer prognosis compared to the solitary form.
- The tumor is hypothesized to originate from heterotopic neuroglial tissue within the leptomeninges.
Implications:
- Highlights the diagnostic challenges of diffuse primary leptomeningeal gliomatosis, often leading to post-mortem diagnosis.
- Emphasizes the aggressive nature and poor prognosis associated with the diffuse subtype.
- Suggests the need for increased awareness among clinicians regarding this rare neoplastic entity presenting as meningitis.