Table 2

Characteristics of patients from population-based evaluation (n = 54) and patients treated with IVE/MTX (n = 26)

Population-based evaluation, n/N (%)IVE/MTX, n/N (%)
Median age, y (range) 57 (28-82) 56 (36-69) 
Female sex 21/54 (39) 9/26 (35) 
Lugano stage   
    I 8/53 (15) 4/26 (15) 
    II 1 8/53 (15) 5/26 (19) 
    II 2 6/53 (11) 2/26 (8) 
    II E 0/53 (0) 3/26 (12) 
    II 1E 0/53 (0) 6/26 (23) 
    II 2E 21/53 (40) 1/26 (4) 
    IV 10/53 (19) 5/26 (19) 
Manchester stage   
    Ia 5/53 (9) 1/26 (4) 
    Ib 3/53 (6) 3/26 (12) 
    IIa 8/53 (15) 5/26 (19) 
    IIb 4/53 (8) 5/26 (19) 
    IIc 17/53 (32) 4/26 (15) 
    III 6/53 (11) 3/26 (12) 
    IV 10/53 (19) 5/26 (19) 
Site of disease in GT   
    Jejunum/ileum 52/54 (96) 22/26 (84) 
    Stomach/duodenum 0/54 (0) 2/26 (8) 
    Duodenojejunal flexure 1/54 (2) 2/26 (8) 
    Ileocecal 1/54 (2) 0/26 (0) 
Bone marrow involvement 2/33 (6) 1/22 (5) 
ECOG greater than 1 46/52 (88) 20/26 (77) 
Presenting features   
    Pain 43/53 (81) 25/26 (96) 
    Lump 3/53 (6) 1/26 (4) 
    Nausea/vomiting 2/53 (38) 18/26 (69) 
    Weight loss 30/53 (57) 19/26 (73) 
    Bowel upset 17/53 (32) 15/26 (58) 
    Perforation 21/53 (40) 8/26 (31) 
    Subacute obstruction 16/53 (30) 10/26 (39) 
Duration of symptoms before diagnosis   
    0-1 mo 17/51 (33) 6/25 (24) 
    1-3 mo 13/51 (26) 7/25 (28) 
    3-6 mo 15/51 (29) 5/25 (20) 
    Longer than 6 mo 6/51 (12) 7/25 (28) 
Presence of celiac disease 48/52 (92) 19/26 (73) 
    Before diagnosis 18/52 (34) 11/26 (42) 
    At diagnosis 30/52 (58) 8/26 (31) 
Diagnosis at   
    Surgery 49/54 (91) 23/26 (89) 
    Endoscopy 4/54 (7) 3/26 (11) 
    Postmortem 1/54 (2) 0/26 (0) 
Abnormal hemoglobin 27/50 (54) 11/26 (42) 
Abnormal WBC count 18/50 (36) 9/26 (35) 
Abnormal kidney function 16/50 (32) 11/25 (44) 
Abnormal liver function 39/51 (76) 16/25 (64) 
Abnormal LDH 11/26 (42) 9/19 (47) 
Population-based evaluation, n/N (%)IVE/MTX, n/N (%)
Median age, y (range) 57 (28-82) 56 (36-69) 
Female sex 21/54 (39) 9/26 (35) 
Lugano stage   
    I 8/53 (15) 4/26 (15) 
    II 1 8/53 (15) 5/26 (19) 
    II 2 6/53 (11) 2/26 (8) 
    II E 0/53 (0) 3/26 (12) 
    II 1E 0/53 (0) 6/26 (23) 
    II 2E 21/53 (40) 1/26 (4) 
    IV 10/53 (19) 5/26 (19) 
Manchester stage   
    Ia 5/53 (9) 1/26 (4) 
    Ib 3/53 (6) 3/26 (12) 
    IIa 8/53 (15) 5/26 (19) 
    IIb 4/53 (8) 5/26 (19) 
    IIc 17/53 (32) 4/26 (15) 
    III 6/53 (11) 3/26 (12) 
    IV 10/53 (19) 5/26 (19) 
Site of disease in GT   
    Jejunum/ileum 52/54 (96) 22/26 (84) 
    Stomach/duodenum 0/54 (0) 2/26 (8) 
    Duodenojejunal flexure 1/54 (2) 2/26 (8) 
    Ileocecal 1/54 (2) 0/26 (0) 
Bone marrow involvement 2/33 (6) 1/22 (5) 
ECOG greater than 1 46/52 (88) 20/26 (77) 
Presenting features   
    Pain 43/53 (81) 25/26 (96) 
    Lump 3/53 (6) 1/26 (4) 
    Nausea/vomiting 2/53 (38) 18/26 (69) 
    Weight loss 30/53 (57) 19/26 (73) 
    Bowel upset 17/53 (32) 15/26 (58) 
    Perforation 21/53 (40) 8/26 (31) 
    Subacute obstruction 16/53 (30) 10/26 (39) 
Duration of symptoms before diagnosis   
    0-1 mo 17/51 (33) 6/25 (24) 
    1-3 mo 13/51 (26) 7/25 (28) 
    3-6 mo 15/51 (29) 5/25 (20) 
    Longer than 6 mo 6/51 (12) 7/25 (28) 
Presence of celiac disease 48/52 (92) 19/26 (73) 
    Before diagnosis 18/52 (34) 11/26 (42) 
    At diagnosis 30/52 (58) 8/26 (31) 
Diagnosis at   
    Surgery 49/54 (91) 23/26 (89) 
    Endoscopy 4/54 (7) 3/26 (11) 
    Postmortem 1/54 (2) 0/26 (0) 
Abnormal hemoglobin 27/50 (54) 11/26 (42) 
Abnormal WBC count 18/50 (36) 9/26 (35) 
Abnormal kidney function 16/50 (32) 11/25 (44) 
Abnormal liver function 39/51 (76) 16/25 (64) 
Abnormal LDH 11/26 (42) 9/19 (47) 

IVE/MTX indicates ifosfamide, vincristine, and etoposide/methotrexate; GT, gastrointestinal tract; ECOG, Eastern Cooperative Oncology Group; WBC, white blood cell; and LDH, lactate dehydrogenase.

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