Primary leptomeningeal lymphoma: International Primary CNS lymphoma collaborative group report

Jennie W. Taylor, Eoin P. Flanagan, Brian P. O'Neill, Tali Siegal, Antonio Omuro, Lisa DeAngelis, Joachim Baehring, Ryo Nishikawa, Fernando Pinto, Marc Chamberlain, Khe Hoang-Xuan, Alberto Gonzalez-Aguilar, Tracy Batchelor, Jean Yves Blay, Agnieszka Korfel, Rebecca A. Betensky, Maria Beatriz S. Lopes, David Schiff

Research output: Contribution to journalArticlepeer-review


Objective: To 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. Methods: The 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. Results: Forty-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. Conclusion: Primary 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.

Original languageEnglish (US)
Pages (from-to)1690-1696
Number of pages7
Issue number19
StatePublished - Nov 5 2013

ASJC Scopus subject areas

  • Clinical Neurology


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