title: Neurolymphomatosis: an International Primary CNS Lymphoma Collaborative Group report.
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.
authors.affiliation: Gaffin Center for Neuro-Oncology and Department of Hematology, Hadassah Hebrew University Medical Center, Jerusalem, Israel.
authors.forename: Sigal
authors.initials: S
authors.lastname: Grisariu
authors.forename: Batia
authors.initials: B
authors.lastname: Avni
authors.forename: Tracy T
authors.initials: TT
authors.lastname: Batchelor
authors.forename: Martin J
authors.initials: MJ
authors.lastname: van den Bent
authors.forename: Felix
authors.initials: F
authors.lastname: Bokstein
authors.forename: David
authors.initials: D
authors.lastname: Schiff
authors.forename: Outi
authors.initials: O
authors.lastname: Kuittinen
authors.forename: Marc C
authors.initials: MC
authors.lastname: Chamberlain
authors.forename: Patrick
authors.initials: P
authors.lastname: Roth
authors.forename: Anatoly
authors.initials: A
authors.lastname: Nemets
authors.forename: Edna
authors.initials: E
authors.lastname: Shalom
authors.forename: Dina
authors.initials: D
authors.lastname: Ben-Yehuda
authors.forename: Tali
authors.initials: T
authors.lastname: Siegal
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002555:Cerebrospinal Fluid / Q000166:cytology; D003299:Cooperative Behavior; D005260:Female; D006801:Humans; D007391:International Cooperation; D007938:Leukemia / Q000401:mortality / Q000473:pathology / Q000628:therapy; D017254:Leukemic Infiltration / Q000401:mortality / Q000473:pathology / Q000628:therapy; D008228:Lymphoma, Non-Hodgkin / Q000401:mortality / Q000473:pathology / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009423:Nervous System Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy; D049268:Positron-Emission Tomography; D012189:Retrospective Studies; D055815:Young Adult