Neurolymphomatosis: An International Primary CNS Lymphoma Collaborative Group report

Sigal Grisariu, Batia Avni, Tracy T. Batchelor, Martin J. Van Den Bent, Felix Bokstein, David Schiff, Outi Kuittinen, Marc C. Chamberlain, Patrick Roth, Anatoly Nemets, Edna Shalom, Dina Ben-Yehuda, Tali Siegal*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

241 Scopus citations


Neurolymphomatosis (NL) is a rare clinical entity. The International Primary CNS Lymphoma Collaborative Group retrospectively analyzed 50 patients assembled from 12 centers in 5 countries over a 16-year period. NL was related to non-Hodgkin lymphoma in 90% and to acute leukemia in 10%. It occurred as the initial manifestation of malignancy in 26% of cases. The affected neural structures included peripheral nerves (60%), spinal nerve roots (48%), cranial nerves (46%), and plexus (40%) with multiple site involvement in 58%. Imaging studies often suggested the diagnosis with 77% positive magnetic resonance imaging, and 84% (16 of 19) positive computed tomography-positron emission tomography studies. Cerebrospinal fluid cytology was positive in 40%, and nerve biopsy confirmed the diagnosis in 23 of 26 (88%). Treatment in 47 patients included systemic chemotherapy (70%), intra-cerebrospinal fluid chemotherapy (49%), and radiotherapy (34%). Response to treatment was observed in 46%. The median overall survival was 10 months, with 12- and 36-month survival proportions of 46% and 24%, respectively. NL is a challenging diagnosis, but contemporary imaging techniques frequently detect the relevant neural invasion. An aggressive multimodality therapy can prevent neurologic deterioration and is associated with a prolonged survival in a subset of patients.

Original languageAmerican English
Pages (from-to)5005-5011
Number of pages7
Issue number24
StatePublished - 17 Jun 2010
Externally publishedYes


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