Table 1.

Initial characteristics and therapy of the 75 PCNSL study patients

VariablePatients
Patients, n 75 
Sex  
 Female 29 (39) 
 Male 46 (61) 
Age, y  
 Median (range) 12.5 (1.25-18.87) 
 <10 26 (35) 
 ≥10 to <15 31 (41) 
 ≥15 18 (24) 
Preexisting disorder  
 Yes 14 (19) 
 No 56 (75) 
 Unknown 5 (6) 
Lansky score  
 <40% 14 (19) 
 ≥40-79% 27 (36) 
 ≥80% 19 (25) 
 Unknown/not applicable 15 (20) 
B symptoms  
 Yes 9 (12) 
 No 59 (79) 
 Unknown 7 (9) 
Cranial nerve palsies  
 Yes 33 (44) 
 No 39 (52) 
 Unknown 3 (4) 
Histopathologic subtype  
 DLBCL 37 (49) 
 Burkitt lymphoma 9 (12) 
 Mature B-NHL NOS 5 (7) 
 ALCL 17 (23) 
 Other NHL 7 (9) 
  BCP LBL, n 
  EN MZL, n 
  PTCL, n 
Localization  
 Intracranial 70 (93) 
 Intraspinal only 2 (3) 
 Leptomeningeal only 3 (4) 
No. of lesions  
 1 36 (48) 
 ≥2 32 (43) 
 Unknown/not applicable 7 (9) 
Initial LDH level, U/L  
 <500 53 (71) 
 ≥500 10 (13) 
 Unknown 12 (16) 
Initial therapy  
 Chemo only 31 (41) 
 Chemo and rituximab 15 (20) 
 Chemo and RT 24 (32) 
 Chemo and rituximab and RT 2 (3) 
 Other 3 (4) 
Therapy  
 Pediatric NHL-type* 57 (76) 
 Adult NHL-type 1 (1) 
 Miscellaneous 17 (23) 
Chemotherapeutic drugs, n§  
 High-dose methotrexate 68 
 High-dose cytarabine 55 
 Anthracyclines 59 
 Alkylating agents 64 
 Intrathecal therapies 69 
RT  
 Yes 26 (35) 
 No 49 (65) 
Stem cell therapy in CR1  
 Autologous 3 (4) 
 Allogeneic 1 (1) 
Rituximab in mature B-NHL 17 (32) 
VariablePatients
Patients, n 75 
Sex  
 Female 29 (39) 
 Male 46 (61) 
Age, y  
 Median (range) 12.5 (1.25-18.87) 
 <10 26 (35) 
 ≥10 to <15 31 (41) 
 ≥15 18 (24) 
Preexisting disorder  
 Yes 14 (19) 
 No 56 (75) 
 Unknown 5 (6) 
Lansky score  
 <40% 14 (19) 
 ≥40-79% 27 (36) 
 ≥80% 19 (25) 
 Unknown/not applicable 15 (20) 
B symptoms  
 Yes 9 (12) 
 No 59 (79) 
 Unknown 7 (9) 
Cranial nerve palsies  
 Yes 33 (44) 
 No 39 (52) 
 Unknown 3 (4) 
Histopathologic subtype  
 DLBCL 37 (49) 
 Burkitt lymphoma 9 (12) 
 Mature B-NHL NOS 5 (7) 
 ALCL 17 (23) 
 Other NHL 7 (9) 
  BCP LBL, n 
  EN MZL, n 
  PTCL, n 
Localization  
 Intracranial 70 (93) 
 Intraspinal only 2 (3) 
 Leptomeningeal only 3 (4) 
No. of lesions  
 1 36 (48) 
 ≥2 32 (43) 
 Unknown/not applicable 7 (9) 
Initial LDH level, U/L  
 <500 53 (71) 
 ≥500 10 (13) 
 Unknown 12 (16) 
Initial therapy  
 Chemo only 31 (41) 
 Chemo and rituximab 15 (20) 
 Chemo and RT 24 (32) 
 Chemo and rituximab and RT 2 (3) 
 Other 3 (4) 
Therapy  
 Pediatric NHL-type* 57 (76) 
 Adult NHL-type 1 (1) 
 Miscellaneous 17 (23) 
Chemotherapeutic drugs, n§  
 High-dose methotrexate 68 
 High-dose cytarabine 55 
 Anthracyclines 59 
 Alkylating agents 64 
 Intrathecal therapies 69 
RT  
 Yes 26 (35) 
 No 49 (65) 
Stem cell therapy in CR1  
 Autologous 3 (4) 
 Allogeneic 1 (1) 
Rituximab in mature B-NHL 17 (32) 

Unless otherwise noted, data are shown as n (%).

BCP, B-cell precursor; Chemo, chemotherapy; CR1, first complete remission; EN MZL, extranodal marginal zone lymphoma; LDH, lactate dehydrogenase; NOS, not otherwise specified; RT, radiotherapy.

*

NHL Berlin-Frankfurt-Münster therapy, n = 30; Lymphomes Malins B therapy, n = 14; Inter–B-NHL therapy, n = 5; Associazione Italiana Ematologia Oncologia Pediatrica linfoma non-Hodgkin (AIEOP LNH) therapy, n = 2; EICNHL therapy, n = 2; Sociedade de Hematologia e Oncologia Pediatrica LNH therapy, n = 1; Japanese Pediatric Leukemia/Lymphoma Study Group B-NHL therapy, n = 2;, Children's Oncology Group AALL1131 trial, n = 1, including 17 of the 26 (65%) patients with RT.

Cancer and Leukemia Group B 50202 trial, n = 1.

Including 9 of the 26 (35%) patients with RT.

§

High-dose-methotrexate (n = 68): <5 g/m2, n = 5; 5 g/m2, n = 38; 8 g/m2, n = 25. High-dose-cytarabine (n = 55): ≤6 g/m2, n = 13; 8 g/m2, n = 1; 12 to 12.5 g/m2, n = 41. Anthracyclines (n = 59) with doxorubicin in 57 patients and a median cumulative dose of 100 mg/m2 (range: 25-240 mg/m2).

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