Abstract
Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare and fatal disease characterized by diffuse infiltration of the leptomeninges by neoplastic glial cells without evidence of tumor in the brain parenchyma or spinal cord. We report a 60-year-old man with PDLG. He suffered transient right hemiparesis and generalized seizures. MRI showed diffuse leptomeningeal thickening and enhancement throughout the brain and spinal cord without any intraaxial involvement. Biopsy resulted in a diagnosis of glioblastoma with methylated MGMT promoter and wild-type IDH1. He underwent craniospinal radiotherapy and temozolomide treatment but despite concomitant adjuvant therapy he died 8 months after initial presentation.
MeSH terms
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Antineoplastic Agents, Alkylating / therapeutic use
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DNA Modification Methylases / genetics
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DNA Repair Enzymes / genetics
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Dacarbazine / analogs & derivatives
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Dacarbazine / therapeutic use
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Fatal Outcome
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Humans
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Magnetic Resonance Imaging
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Male
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Meningeal Neoplasms / diagnosis*
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Meningeal Neoplasms / genetics
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Meningeal Neoplasms / pathology*
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Meningeal Neoplasms / therapy
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Methylation
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Middle Aged
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Neoplasms, Neuroepithelial / diagnosis*
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Neoplasms, Neuroepithelial / genetics
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Neoplasms, Neuroepithelial / pathology*
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Neoplasms, Neuroepithelial / therapy
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Promoter Regions, Genetic
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Radiotherapy / methods
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Temozolomide
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Tumor Suppressor Proteins / genetics
Substances
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Antineoplastic Agents, Alkylating
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Tumor Suppressor Proteins
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Dacarbazine
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DNA Modification Methylases
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MGMT protein, human
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DNA Repair Enzymes
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Temozolomide