Table 1

Patient characteristics and clinical features of neurolymphomatosis

CharacteristicGroup A: literature review with MGH case series (1972-2000), N = 72Group B: literature review, case reports (2001-2008), N = 44Group C: current IPCG case series (1993-2008), N = 50
Sex    
    Male 39 (54) 26 (59) 30 (60) 
    Female 33 (46) 16 (36) 20 (40) 
    Not reported  2 (4.5)  
Median age, y (range) 63 (18-84) 56 (16-71) 55.5 (18-80) 
NL as an extranodal site of systemic lymphoma 29 (40) 27 (61) 33 (66) 
NL as the presentation of malignancy NA 13 (29.5) 13 (26) 
Malignant cell type    
    B cells 59 (82) 29 (66) 41 (82) 
    T cells 4 (5) 11 (25) 5 (10) 
    NK cells  1 (2)  
    Not classified 9 (13) 3 (6.8) 4 (8) 
Parenchymal brain involvement 19 (26) NA 11 (22), PCNSL 
Affected neural structures*    
    Peripheral nerves 46 (64) 9 (20) 30 (60) 
    Spinal nerves 47 (65) 14 (32) 24 (48) 
    Neural plexus 23 (32) 15 (34) 20 (40) 
    Cranial nerves 37 (51) 15 (34) 23 (46) 
Painful neuropathy 34 (47) 25 (57) 38 (76) 
Type of neuropathy    
    Pure motor NA 7 (16) 11 (22) 
    Pure sensory NA 8 (18) 1 (2) 
    Sensorimotor NA 23 (52) 36 (72) 
    Not reported  6 (14) 2 (4) 
CharacteristicGroup A: literature review with MGH case series (1972-2000), N = 72Group B: literature review, case reports (2001-2008), N = 44Group C: current IPCG case series (1993-2008), N = 50
Sex    
    Male 39 (54) 26 (59) 30 (60) 
    Female 33 (46) 16 (36) 20 (40) 
    Not reported  2 (4.5)  
Median age, y (range) 63 (18-84) 56 (16-71) 55.5 (18-80) 
NL as an extranodal site of systemic lymphoma 29 (40) 27 (61) 33 (66) 
NL as the presentation of malignancy NA 13 (29.5) 13 (26) 
Malignant cell type    
    B cells 59 (82) 29 (66) 41 (82) 
    T cells 4 (5) 11 (25) 5 (10) 
    NK cells  1 (2)  
    Not classified 9 (13) 3 (6.8) 4 (8) 
Parenchymal brain involvement 19 (26) NA 11 (22), PCNSL 
Affected neural structures*    
    Peripheral nerves 46 (64) 9 (20) 30 (60) 
    Spinal nerves 47 (65) 14 (32) 24 (48) 
    Neural plexus 23 (32) 15 (34) 20 (40) 
    Cranial nerves 37 (51) 15 (34) 23 (46) 
Painful neuropathy 34 (47) 25 (57) 38 (76) 
Type of neuropathy    
    Pure motor NA 7 (16) 11 (22) 
    Pure sensory NA 8 (18) 1 (2) 
    Sensorimotor NA 23 (52) 36 (72) 
    Not reported  6 (14) 2 (4) 

Values are no. (%), except for age (y). Group A consists of a retrospective case series of 25 patients diagnosed in Massachusetts General Hospital (MGH) and literature review of additional 47 cases that were published together. Group B is based on literature review.4,7-41  Group C is the current case series.

NA indicates not available; and PCNSL, primary central nervous system lymphoma.

*

Involvement of multiple sites was common.

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