International study on low-grade primary central nervous system lymphoma.
OBJECTIVE
The aim of this study was to characterize the clinical presentation, course, and outcome of low-grade primary central nervous system lymphoma.


METHODS
Cases were assessed in a retrospective series collected from 18 cancer centers in 5 countries.


RESULTS
Forty patients (18 men, 22 women; median age, 60 years [range, 19-78]) were identified. Involvement of a cerebral hemisphere or deeper brain structures was seen in 37 patients, only leptomeningeal involvement in 2 patients, and spinal cord disease in 1 patient. Chemotherapy/radiotherapy was conducted in 15 patients, radiotherapy alone in 12, chemotherapy alone in 10, and tumor resection alone in 2, whereas 1 patient received no treatment. The median progression-free, disease-specific, and overall survival were 61.5 (range, 0-204), 130 (range, 1-204), and 79 (range, 1-204) months, respectively. Only age 60 years or older was associated with shorter progression-free (p = 0.009), disease-specific (p = 0.015), and overall survival (p = 0.001) in multivariate analysis.


CONCLUSIONS
Low-grade primary central nervous system lymphoma differs from the high-grade subtype in its pathological, clinical, and radiological features. It has a better long-term outcome than primary central nervous system lymphoma in general with age 60 years or older adversely affecting survival.
Department of Hematology, Charité-Universitätsmedizin Berlin, Campus Benjamin Franklin, Berlin, Germany. kristophe.jahnke@charite.de
Kristoph
K
Jahnke
Agnieszka
A
Korfel
Brian Patrick
BP
O'Neill
Jean-Yves
JY
Blay
Lauren E
LE
Abrey
Peter
P
Martus
Philip M P
PM
Poortmans
Tamara N
TN
Shenkier
Tracy T
TT
Batchelor
Edward A
EA
Neuwelt
Jeffrey J
JJ
Raizer
David
D
Schiff
Hendrik
H
Pels
Ulrich
U
Herrlinger
Harald
H
Stein
Eckhard
E
Thiel
D000367:Age Factors; D000368:Aged; D001921:Brain / Q000473:pathology; D016543:Central Nervous System Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D006801:Humans; D007391:International Cooperation; D008223:Lymphoma / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome