Table 2

Diagnostic modalities and response to treatment of neurolymphomatosis

Diagnostic/treatment modalityGroup 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
Imaging    
    CT NA 3/11 (27) 7/11 (64) 
    MRI 28/40 (70) 28/35 (80) 36/47 (77) 
    FDG-PET NA 19/21 (90) 16/19 (84) 
CSF cytology 21/52 (40) 10/24 (42) 18/45 (40) 
CSF PCR gene rearrangement NA 2/2 (100) 3/11 (27) 
Biopsy of affected nerve 24/30 (80) 19/21 (90) 23/26 (88) 
Diagnosis established only by autopsy 33 (46) 2 (5) 4 (8) 
No. of patients treated for NL* 43 (60) 34 (77) 47 (94) 
    IV HD-MTX 5/43 (12) 8/34 (23.5) 23/47 (49) 
    Intra-CSF chemotherapy 15/43 (35) 14/34 (41) 23/47 (49) 
    Radiotherapy 10/43 (23) 17/34 (50) 16/47 (34) 
Response rate 31/43 (72) 20/34 (58) 16/35 (46) 
Diagnostic/treatment modalityGroup 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
Imaging    
    CT NA 3/11 (27) 7/11 (64) 
    MRI 28/40 (70) 28/35 (80) 36/47 (77) 
    FDG-PET NA 19/21 (90) 16/19 (84) 
CSF cytology 21/52 (40) 10/24 (42) 18/45 (40) 
CSF PCR gene rearrangement NA 2/2 (100) 3/11 (27) 
Biopsy of affected nerve 24/30 (80) 19/21 (90) 23/26 (88) 
Diagnosis established only by autopsy 33 (46) 2 (5) 4 (8) 
No. of patients treated for NL* 43 (60) 34 (77) 47 (94) 
    IV HD-MTX 5/43 (12) 8/34 (23.5) 23/47 (49) 
    Intra-CSF chemotherapy 15/43 (35) 14/34 (41) 23/47 (49) 
    Radiotherapy 10/43 (23) 17/34 (50) 16/47 (34) 
Response rate 31/43 (72) 20/34 (58) 16/35 (46) 

Values are no. positive/no. of tests (%). 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; PCR, polymerase chain reaction; and IV HD-MTX, intravenous high-dose methotrexate.

*

Some patients were treated by chemotherapy other than high-dose methotrexate.

Includes complete and partial response by clinical improvement or by posttreatment imaging.

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