ArticleNeurology2013
Primary leptomeningeal lymphoma: International Primary CNS Lymphoma Collaborative Group report.
Article in Neurology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers.
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Who cites it
38 citing papers in PubMed, 93 citations in OpenAlex.
- Dural Follicular Non-Hodgkin Lymphoma Mimicking a Chronic Subdural Hematoma: A Case Report and Literature Review.Cureus · 2026Article
- Rashless varicella-zoster virus encephalitis diagnosed by metagenomic next-generation sequencing: two case reports.BMC neurology · 2026Article
- Extraparenchymal Brain Lesions in Anti-NMDAR Encephalitis Leading to Primary Leptomeningeal Lymphoma: A Rare Evolution with Diagnostic Implications.Internal medicine (Tokyo, Japan) · 2026Article
- Primary Central Nervous System Lymphoma: A Case Report of the Neurological Great Mimicker.Cureus · 2025Article
- Primary subdural lymphoma mimicking chronic subdural hematoma: a case report and a narrative review of some recent similar cases in the literature.International journal of emergency medicine · 2025Article
- Article
- Efficacy of thiotepa-conditioned autologous stem-cell transplantation as consolidation therapy for primary leptomeningeal malignant lymphoma: a case report and review of literature.Annals of hematology · 2024Review
- Spatial transcriptomics analysis identifies a tumor-promoting function of the meningeal stroma in melanoma leptomeningeal disease.Cell reports. Medicine · 2024Article
- Diagnostics and treatment delay in primary central nervous system lymphoma: What the neurosurgeon should know.Acta neurochirurgica · 2024Review
- Spatial transcriptomics analysis identifies a unique tumor-promoting function of the meningeal stroma in melanoma leptomeningeal disease.bioRxiv : the preprint server for biology · 2023Article
- The Spectrum of Headache in Leptomeningeal Metastases: A Comprehensive Review with Clinical Management Guidelines.Current pain and headache reports · 2023Review
- Pretreatment findings on magnetic resonance imaging in primary central nervous system lymphoma may predict overall survival duration.The neuroradiology journal · 2023Article
- Primary Central Nervous System Lymphoma Presenting as Isolated Multiple Cranial Neuropathies: An Extremely Rare Case.Cureus · 2023Article
- Leptomeningeal Metastasis: A Review of the Pathophysiology, Diagnostic Methodology, and Therapeutic Landscape.Current oncology (Toronto, Ont.) · 2023Review
- Primary dural lymphoma: Case report.Annals of medicine and surgery (2012) · 2022Article
- Different facets of intracranial central nervous system lymphoma and its imaging mimics.Journal of clinical imaging science · 2022Article
- Central Nervous System Lymphoma: Novel Therapies.Current treatment options in oncology · 2022Review
- Article
- Treatment of Primary CNS Lymphoma: Maximizing Clinical Benefit, Minimizing Neurotoxicity.Current oncology reports · 2021Review
- Primary leptomeningeal lymphoma masquerading as infectious tubercular meningitis.BMJ case reports · 2021Article
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Authors and funding
18 authors at 10 institutions in 5 countries.
Funding
Abstract
objectiveTo evaluate clinical presentation, optimal diagnostic evaluation and treatment, and outcome in primary leptomeningeal lymphoma, a rare form of primary CNS lymphoma without parenchymal or systemic involvement.
methodsThe International Primary CNS Lymphoma Collaborative Group, a multidisciplinary group of physicians with a particular interest in primary CNS lymphoma, retrospectively identified cases of lymphoma isolated to the leptomeninges as diagnosed by CSF cytology, flow cytometry, or biopsy, without systemic or parenchymal brain/spinal cord lymphoma or immunodeficiency.
resultsForty-eight patients were identified, with median age at diagnosis of 51 years and median Eastern Cooperative Oncology Group performance status of 2. Presenting symptoms were multifocal in 68%. Leptomeningeal enhancement was seen in 74% and CSF profile was abnormal in all cases. CSF cytology detected malignant lymphocytes in 67%. Flow cytometry identified monoclonal population in 80%, as did receptor gene rearrangement studies in 71%. Sixty-two percent had B-cell lymphoma, 19% T-cell, and 19% unclassified. Treatment varied and included fractionated radiotherapy (36%), systemic chemotherapy (78%), and intra-CSF chemotherapy (66%), with 66% receiving ≥ 2 modalities. Seventy-one percent had a favorable clinical response; ultimately, 44% received salvage treatment. Median overall survival was 24 months, with 11 patients still alive at 50 months follow-up.
conclusionPrimary leptomeningeal lymphoma is a rare form of primary CNS lymphoma. Patients usually present with multifocal symptoms, with evidence of leptomeningeal enhancement and diagnostic CSF analysis. Although treatment is highly variable, patients have a better prognosis than previously reported and a subset may be cured.
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