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Updated: May 2, 2026

Author Spotlight: Unveiling Cellular Functions and Potential Clinical Implications of Leptomeningeal Lymphatic Endothelial Cells
Published on: September 8, 2023
[A case of intracranial primary leptomeningeal lymphoma]
Kyohei Kin1, Yasuhiro Ono, Yoshio Hisamatsu
1Department of Neurosurgery, Kagawa Prefectural Central Hospital.
Abstract:
Primary leptomeningeal lymphoma(PLML)is a neoplastic meningitis of lymphomatous origin without parenchymal central nervous system(CNS)disease or a systemic tumor. We report a case of PLML that presented with epileptic seizure, and review relevant literature. A 27-year-old man was brought to the emergency department with an epileptic seizure. Two months later, he was again brought to the emergency department with an epileptic seizure. MRI showed enhanced lesions on the surface of the right cerebellar hemisphere, right parietal sulci, and interhemispheric surface of the frontal lobes. We performed an open biopsy and diagnosed the patient with diffuse large B-cell lymphoma of the leptomeninges on the basis of histological findings. The patient was initially treated with chemotherapy including high-dose methotrexate(MTX). Because remission was not achieved by chemotherapy, the patient was treated with whole-brain radiation therapy. After onset, the patient survived for 2 years without recurrence. PLML is a particularly rare type of primary CNS lymphoma. The outcome of PLML, compared with general primary CNS lymphoma, is reported to be very poor because chemotherapy including MTX is ineffective.
Insights
Primary leptomeningeal lymphoma (PLML) is a rare CNS malignancy. This case highlights a successful treatment approach combining chemotherapy and radiation therapy, achieving a 2-year remission in a patient with diffuse large B-cell lymphoma.
Area of Science:
- Neurology
- Oncology
- Pathology
Background:
- Primary leptomeningeal lymphoma (PLML) is a rare neoplastic meningitis without CNS parenchymal disease or systemic tumor.
- It presents a diagnostic challenge due to its rarity and potential for varied clinical manifestations.
Observation:
- A 27-year-old male presented with recurrent epileptic seizures.
- MRI revealed enhanced leptomeningeal lesions on the cerebellar, parietal, and frontal lobes.
- Histopathological analysis confirmed diffuse large B-cell lymphoma of the leptomeninges.
Findings:
- Initial chemotherapy with high-dose methotrexate (MTX) failed to induce remission.
- Whole-brain radiation therapy was administered as a subsequent treatment.
- The patient achieved a 2-year disease-free survival after combined modality treatment.
Implications:
- PLML, often associated with poor outcomes and resistance to chemotherapy, may be effectively managed with radiation therapy.
- This case suggests a potential therapeutic role for radiation in refractory PLML.
- Further research is warranted to optimize treatment strategies for this rare condition.
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