Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-35509
Grisariu, S; Avni, B; Batchelor, T T; van den Bent, M J; Bokstein, F; Schiff, D; Kuittinen, O; Chamberlain, M C; Roth, P; Nemets, A; Shalom, E; Ben-Yehuda, D; Siegal, T (2010). Neurolymphomatosis: an International Primary CNS Lymphoma Collaborative Group report. Blood, 115(24):5005-5011.
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.
|Item Type:||Journal Article, refereed, original work|
|Communities & Collections:||04 Faculty of Medicine > University Hospital Zurich > Clinic for Neurology|
|DDC:||610 Medicine & health|
|Deposited On:||01 Oct 2010 15:25|
|Last Modified:||27 Nov 2013 19:15|
|Additional Information:||This research was originally published in Blood, 17 June 2010, Vol. 115, No. 24, pp. 5005-5011. Copyright by the American Society of Hematology|
|Citations:||Web of Science®. Times Cited: 27|
Scopus®. Citation Count: 37
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