Primary leptomeningeal lymphoma: International Primary CNS Lymphoma Collaborative Group report

Neurology. 2013 Nov 5;81(19):1690-6. doi: 10.1212/01.wnl.0000435302.02895.f3. Epub 2013 Oct 9.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Central Nervous System Neoplasms / cerebrospinal fluid
  • Central Nervous System Neoplasms / diagnosis*
  • Central Nervous System Neoplasms / mortality
  • Central Nervous System Neoplasms / therapy*
  • Child
  • Female
  • Humans
  • Kaplan-Meier Estimate
  • Lymphoma / cerebrospinal fluid
  • Lymphoma / diagnosis*
  • Lymphoma / therapy*
  • Magnetic Resonance Imaging
  • Male
  • Meningeal Neoplasms / cerebrospinal fluid
  • Meningeal Neoplasms / diagnosis*
  • Meningeal Neoplasms / mortality
  • Meningeal Neoplasms / therapy*
  • Middle Aged
  • Proportional Hazards Models
  • Retrospective Studies
  • Treatment Outcome
  • Young Adult