Lymphomatosis cerebri: A rare form of primary central nervous system lymphoma. Analysis of 7 cases and systematic review of the literature

Cristina Izquierdo, Roser Velasco, Noemí Vidal, Juan José Sánchez, Andreas A. Argyriou, Sarah Besora, Francesc Graus, Jordi Bruna

Research output: Contribution to journalReview articleResearchpeer-review

33 Citations (Scopus)


© 2015 The Author(s). Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. Background. Primary central nervous system lymphomas may present as diffuse, nonenhancing infiltrative lesions. This rare variant is termed lymphomatosis cerebri (LC). We did a systematic review and analysis of the literature, adding our own cases, to better characterize LC in order to improve early diagnosis and treatment. Methods. PubMed, ISI Web of Knowledge, and hospital databases were reviewed. Information was extracted regarding demographic, clinical, histological, cerebrospinal fluid (CSF), neuroimaging, and treatment variables. The impact of single parameters on overall survival (OS) was determined by applying univariate and multivariate analyses. Results. Forty-two patients were included (median age: 58 y; range: 28-80 y). At consultation, 52% of patients had a poor KPS. The most common presenting symptom was cognitive decline (59.5%). Imaging studies showed supratentorial and infratentorial infiltration in 55% of patients and bilateral hemispheric involvement in 95%. CSF pleocytosis was present in 51.5% of the patients. Median time to diagnosis was 4.5 (range: 1-30) months, and the diagnosis was not established until autopsy for 33% of patients. The median OS was 2.95 (range: 0.33-56) months; however, those patients who received methotrexate had a median OS of 13.8 (range: 0.7-56) months. Analysis identified KPS ≥ 70 (HR: 0.32; 95% CI: 0.114-0.894; P =. 03) and treatment with methotrexate (HR: 0.19; 95% CI: 0.041-0.886; P =. 034) as independent favorable prognostic factors, whereas T-cell lymphoma was independently related with a worse outcome (HR: 6.62; 95% CI: 1.317-33.316; P =. 022). Conclusions. LC is a misdiagnosed entity associated with considerable diagnostic delay. MRI evidence of bilateral hemispheric involvement and CSF pleocytosis should be alerts for this diagnosis. Treatment with methotrexate-based chemotherapy must be considered, especially for patients with good KPS.
Original languageEnglish
Pages (from-to)707-715
Issue number5
Publication statusPublished - 1 May 2016


  • diffuse infiltrative lesion
  • leukoencephalopathy
  • lymphomatosis cerebri
  • primary central nervous system lymphoma


Dive into the research topics of 'Lymphomatosis cerebri: A rare form of primary central nervous system lymphoma. Analysis of 7 cases and systematic review of the literature'. Together they form a unique fingerprint.

Cite this