Neurolymphomatosis: an International Primary CNS Lymphoma Collaborative Group report

Author:

Grisariu Sigal1,Avni Batia1,Batchelor Tracy T.2,van den Bent Martin J.3,Bokstein Felix4,Schiff David5,Kuittinen Outi6,Chamberlain Marc C.7,Roth Patrick8,Nemets Anatoly9,Shalom Edna1,Ben-Yehuda Dina1,Siegal Tali1

Affiliation:

1. Gaffin Center for Neuro-Oncology and Department of Hematology, Hadassah Hebrew University Medical Center, Jerusalem, Israel;

2. Massachusetts General Hospital Cancer Center, Boston;

3. Neuro-Oncology Unit, Daniel den Hoed Cancer Center/Erasmus University Medical Center, Rotterdam, The Netherlands;

4. Department of Oncology, Sourasky Medical Center, Tel Aviv, Israel;

5. Neuro-Oncology Center, University of Virginia, Charlottesville;

6. Department of Oncology, Oulu University Hospital, Oulu, Finland;

7. Department of Neurology, University of Washington, Seattle;

8. Department of Neurology, University Hospital Zurich, Zurich, Switzerland; and

9. Department of Hematology, Barzilai Medical Center, Ashkelon, Israel

Abstract

Abstract 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.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Reference41 articles.

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