International Study on Low-Grade Primary Central Nervous System Lymphoma

Ann Neurol. 2006 May;59(5):755-62. doi: 10.1002/ana.20804.


Objective: The aim of this study was to characterize the clinical presentation, course, and outcome of low-grade primary central nervous system lymphoma.

Methods: Cases were assessed in a retrospective series collected from 18 cancer centers in 5 countries.

Results: Forty patients (18 men, 22 women; median age, 60 years [range, 19-78]) were identified. Involvement of a cerebral hemisphere or deeper brain structures was seen in 37 patients, only leptomeningeal involvement in 2 patients, and spinal cord disease in 1 patient. Chemotherapy/radiotherapy was conducted in 15 patients, radiotherapy alone in 12, chemotherapy alone in 10, and tumor resection alone in 2, whereas 1 patient received no treatment. The median progression-free, disease-specific, and overall survival were 61.5 (range, 0-204), 130 (range, 1-204), and 79 (range, 1-204) months, respectively. Only age 60 years or older was associated with shorter progression-free (p = 0.009), disease-specific (p = 0.015), and overall survival (p = 0.001) in multivariate analysis.

Interpretation: Low-grade primary central nervous system lymphoma differs from the high-grade subtype in its pathological, clinical, and radiological features. It has a better long-term outcome than primary central nervous system lymphoma in general with age 60 years or older adversely affecting survival.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Age Factors
  • Aged
  • Brain / pathology
  • Central Nervous System Neoplasms / drug therapy
  • Central Nervous System Neoplasms / pathology*
  • Central Nervous System Neoplasms / radiotherapy
  • Combined Modality Therapy
  • Disease Progression
  • Female
  • Humans
  • International Cooperation
  • Lymphoma / drug therapy
  • Lymphoma / pathology*
  • Lymphoma / radiotherapy
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Retrospective Studies
  • Survival Analysis
  • Treatment Outcome