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Diffuse primary leptomeningeal gliomatosis
G Davila1, C Duyckaerts, J P Lazareth
1Department of Neurology, Hôpital de la Salpétrière, Paris, France.
Journal of Neuro-Oncology
|January 1, 1993
Summary
A rare case of leptomeningeal gliomatosis, mimicking carcinomatosis, occurred in a 38-year-old patient. Autopsy revealed a previously unknown hippocampal astrocytoma as the origin.
Area of Science:
- Neurology
- Neuro-oncology
- Pathology
Background:
- Leptomeningeal carcinomatosis (LC) presents with neurological deficits and is diagnosed via cerebrospinal fluid (CSF) cytology.
- Unknown primary tumors are a common challenge in diagnosing LC.
Observation:
- A 38-year-old patient presented with multiple cranial nerve palsies, seizures, and altered consciousness.
- CSF analysis revealed tumor cells, leading to a diagnosis of leptomeningeal carcinomatosis from an unknown primary.
- Intrathecal methotrexate and cranial radiation therapy were ineffective.
Findings:
- Autopsy identified widespread leptomeningeal gliomatosis.
- The gliomatosis originated from a previously undetected astrocytoma in the hippocampus.
Implications:
- This case highlights the importance of considering primary brain tumors, particularly hippocampal gliomas, in the differential diagnosis of leptomeningeal disease.
- It underscores the challenges in diagnosing and treating leptomeningeal gliomatosis when the primary source is occult.
- Further research into diagnostic markers and targeted therapies for primary leptomeningeal gliomatosis is warranted.