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Aggressive treatment with complete remission in primary diffuse leptomeningeal gliomatosis: A case report

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Abstract

Primary leptomeningeal gliomatosis is rare, and the diffuse form (PLDG) is even more unusual. The following report is an example. A 17 year-old man developed a syndrome characterized by extensive basal and chronic spinal meningitis. Routine biological tests showed elevated levels of CSF proteins, and moderate mononuclear pleocytosis, with no direct evidence of neoplasia, leading to a diagnosis of chronic meningitis. A second meningeal biopsy, guided by MRI and performed in the left frontal region, led to the specific diagnosis of primary diffuse leptomeningeal gliomatosis. Treatment including ventricular and lumbar shunting, a course of cortico-spinal radiation, and three courses of an eight-drug systemic chemotherapy with intrathecal methotrexate lead to complete remission over 15 months. We believe that this is the first report of such a remission in the literature.

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Authors and Affiliations

  1. Service de Neurochirurgie (PB, RD), de Neuroradiologie (FGB), d'Oncologie Médicale (GC), de Radiothérapie (TS) et de Neurologie (BL), Centre Hospitalier Universitaire, Saint-Etienne

    P. Beauchesne, R. Duthel, F.G. Barral, G. Clavreul, T. Schmitt & B. Laurent

  2. Laboratoire d'Anatomie Pathologique, place Plaisance, Saint-Chamond, France

    J. Pialat

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  1. P. Beauchesne
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  2. J. Pialat
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  3. R. Duthel
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  4. F.G. Barral
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  5. G. Clavreul
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  6. T. Schmitt
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  7. B. Laurent
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Beauchesne, P., Pialat, J., Duthel, R. et al. Aggressive treatment with complete remission in primary diffuse leptomeningeal gliomatosis: A case report. J Neurooncol 37, 161–167 (1998). https://doi.org/10.1023/A:1005888319228

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