Background
Methods
Patients and tumor localization
Clinical data
Statistical analysis
Results
Primary site of involvement
Primary site | Total cases (n = 581) | B-cell lymphoma (n = 504) | T-cell lymphoma (n = 77) | P value* |
---|---|---|---|---|
Small intestine | ||||
Duodenum | 31 (5.3) | 25 (5.0) | 6 (7.8) | 0.02 |
Jejunum | 34 (5.9) | 22 (4.4) | 12 (15.6) | |
Ileum | 97 (16.7) | 84 (16.7) | 13 (16.9) | |
Ileocecal region | 231 (39.8) | 221 (43.8) | 10 (13.0) | < 0.001 |
Large intestine | ||||
Ascending/transverse colon | 87 (15.0) | 70 (13.9) | 17 (22.1) | 0.14 |
Descending/sigmoid colon | 12 (2.1) | 11 (2.2) | 1 (1.3) | |
Rectum | 26 (4.5) | 25 (5.0) | 1 (1.3) | |
Multiple intestinal Involvement | 63 (10.8) | 46 (9.1) | 17 (22.1) | 0.002 |
Characteristics of patients
Characteristics | Total cases (n = 581) | Small intestine (n = 162) | Ileocecal region (n = 231) | Large intestine (n = 125) | Multiple intestinal involvement (n = 63) | P value | |
---|---|---|---|---|---|---|---|
Age (years) | ≤ 60 | 356 (61.3) | 100 (61.7) | 146 (63.2) | 77 (61.6) | 33 (52.4) | 0.479 |
> 60 | 225 (38.7) | 62 (38.3) | 85 (36.8) | 48 (38.4) | 30 (47.6) | ||
Sex | Male | 367 (63.2) | 108 (66.7) | 146 (63.2) | 73 (58.4) | 40 (63.5) | 0.557 |
Female | 214 (36.8) | 54 (33.3) | 85 (36.8) | 52 (41.6) | 23 (36.5) | ||
Performance status | ECOG 0/1 | 490 (84.3) | 135 (83.9) | 197 (85.3) | 104 (83.2) | 54 (85.7) | 0.942 |
ECOG ≥ 2 | 90 (15.5) | 26 (16.0) | 34 (14.7) | 21 (16.8) | 9 (14.3) | ||
Missing | 1 (0.2) | 1 (0.1) | |||||
Serum LDH level | Normal | 355 (61.1) | 92 (56.8) | 152 (65.8) | 77 (61.6) | 34 (54.0) | 0.086 |
Increased | 210 (36.1) | 67 (41.4) | 71 (30.7) | 43 (34.4) | 29 (46.0) | ||
Missing | 16 (2.8) | 3 (1.8) | 8 (3.5) | 5 (4.0) | |||
B symptoms | Absent | 459 (79.0) | 125 (77.2) | 185 (80.1) | 103 (82.4) | 46 (73.0) | 0.441 |
Present | 120 (20.7) | 36 (22.2) | 45 (19.5) | 22 (17.6) | 17 (27.0) | ||
Missing | 2 (0.3) | 1 (0.6) | 1 (0.4) | ||||
Intestinal symptoms | Obstruction | 96 (16.5) | 25 (15.4) | 47 (20.3) | 14 (11.2) | 10 (15.9) | 0.267 |
Bleeding | 13 (2.2) | 2 (1.2) | 8 (3.5) | 3 (2.4) | 0 (0.0) | ||
Perforation | 25 (4.3) | 8 (4.9) | 13 (5.6) | 3 (2.4) | 1 (1.6) | ||
Extranodal involvement | < 2 | 417 (71.8) | 105 (64.8) | 179 (77.5) | 103 (82.4) | 30 (47.6) | < 0.001 |
≥ 2 | 155 (26.7) | 55 (34.0) | 47 (20.3) | 21 (16.8) | 32 (50.8) | ||
Missing | 9 (1.5) | 2 (1.2) | 5 (2.2) | 1 (1.0) | 1 (1.6) | ||
IPI | L/LI | 277/151 (75.4) | 76/40 (71.6) | 129/48 (76.6) | 59/38 (77.6) | 13/25 (60.3) | < 0.001 |
HI/H | 87/53 (22.4) | 24/20 (27.2) | 33/15 (20.8) | 16/8 (19.2) | 14/10 (38.1) | ||
Missing | 13 (2.2) | 2 (1.2) | 6 (2.6) | 4 (3.2) | 1 (1.6) | ||
Lugano stage | I/II | 139/264 (71.1) | 37/73 (67.9) | 54/126 (77.9) | 43/54 (77.6) | 5/21 (41.3) | < 0.001 |
IV | 168 (28.9) | 52 (32.1) | 51 (22.1) | 28 (22.4) | 37 (58.7) | ||
BM invasion | Absent | 494 (85.0) | 131 (80.9) | 199 (86.1) | 111 (88.8) | 53 (84.1) | 0.300 |
Present | 43 (7.4) | 17 (10.5) | 13 (5.6) | 6 (4.8) | 7 (11.1) | ||
ND | 44 (7.6) | 14 (8.6) | 19 (8.2) | 8 (6.4) | 3 (4.8) | ||
Immunophenotype | B-cell | 504 (86.7) | 131 (80.9) | 221 (95.7) | 106 (84.8) | 46 (73.0) | < 0.001 |
T-cell | 77 (13.3) | 31 (19.1) | 10 (4.3) | 19 (15.2) | 17 (27.0) |
Histological distribution
Comparison of histologic subtypes
Characteristics | DLBCL No. (%) | MALT No. (%) | BL No. (%) | MCL No. (%) | FL No. (%) | PTCL-U No. (%) | EATL No. (%) | ENKTL No. (%) | P value |
---|---|---|---|---|---|---|---|---|---|
Number of cases | 386 | 61 | 31 | 19 | 7 | 34 | 25 | 18 | |
Median age (range) | 56 (15-92) | 55 (15-80) | 47 (15-78) | 60 (42-78) | 52 (39-81) | 49 (15-78) | 51 (23-75) | 47 (32-72) | 0.002 |
Age > 60, % | 160 (41.5) | 22 (36.1) | 7 (22.6) | 10 (52.6) | 3 (42.9) | 11 (32.4) | 7 (28.0) | 5 (27.8) | 0.246 |
Male, % | 240 (62.2) | 32 (52.5) | 25 (80.6) | 13 (68.4) | 4 (57.1) | 24 (70.6) | 17 (68.0) | 12 (66.7) | 0.273 |
Performance status ≥ 2, % | 60 (15.6) | 6 (9.8) | 5 (16.1) | 0 (0.0) | 1 (14.3) | 9 (26.5) | 6 (24.0) | 3 (16.7) | 0.218 |
Lugano stage IV, % | 94 (24.4) | 7 (11.5) | 17 (54.8) | 15 (78.9) | 3 (42.9) | 15 (44.1) | 8 (32.0) | 9 (50.0) | < 0.001 |
Increased serum LDH, % | 150 (39.9) | 5 (8.5) | 23 (76.7) | 4 (21.1) | 1 (14.3) | 12 (36.4) | 8 (34.8) | 7 (38.9) | < 0.001 |
Presence of B symptoms, % | 75 (19.5) | 7 (11.5) | 7 (22.6) | 3 (15.8) | 1 (14.3) | 12 (35.3) | 9 (36.0) | 6 (35.3) | 0.048 |
Extranodal involvement ≥ 2, % | 93 (24.3) | 3 (5.4) | 18 (58.1) | 9 (47.4) | 2 (28.6) | 10 (29.4) | 10 (41.7) | 10 (55.6) | < 0.001 |
IPI HI/H, % | 93 (24.5) | 5 (8.9) | 15 (48.4) | 7 (36.8) | 1 (14.3) | 9 (26.5) | 6 (26.1) | 4 (22.2) | 0.008 |
Bone marrow invasion, % | 20 (5.2) | 4 (6.6) | 7 (22.6) | 5 (26.3) | 0 (0.0) | 3 (8.8) | 3 (12.0) | 1 (5.6) | < 0.001 |
Intestinal obstruction, % | 69 (17.8) | 7 (11.5) | 6 (19.4) | 0 (0.0) | 2 (28.5) | 4 (11.8) | 4 (16.0) | 4 (22.2) | 0.398 |
Bleeding, % | 8 (2.0) | 2 (3.3) | 1 (3.2) | 0 (0.0) | 0 (0.0) | 1 (2.9) | 1 (4.0) | 0 (0.0) | 0.964 |
Perforation, % | 19 (4.9) | 1 (1.6) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 2 (5.9) | 3 (12.0) | 0 (0.0) | 0.194 |
Small intestine, % | 104 (26.9) | 14 (23.0) | 11 (35.5) | 0 (0.0) | 2 (28.6) | 13 (38.2) | 10 (40.0) | 8 (44.4) | < 0.001 |
Ileocecal region, % | 187 (48.4) | 19 (31.1) | 9 (29.0) | 3 (15.8) | 3 (42.9) | 7 (20.6) | 2 (8.0) | 1 (5.6) | < 0.001 |
Large intestine, % | 73 (18.9) | 21 (34.4) | 5 (16.1) | 5 (26.3) | 2 (28.6) | 5 (14.7) | 10 (40.0) | 4 (22.2) | < 0.001 |
Multiple intestinal lesions, % | 22 (5.7) | 7 (11.5) | 6 (19.4) | 11 (57.9) | 0 (0.0) | 9 (26.5) | 3 (12.0) | 5 (27.8) | < 0.001 |
Treatments and outcomes
Characteristics | Total cases (n = 581) | Small intestine (n = 162) | Ileocecal region (n = 231) | Large intestine (n = 125) | Multiple intestinal involvement (n = 63) |
---|---|---|---|---|---|
Treatment* | |||||
Chemotherapy | 521 (89.7%) | 143 (88.3%) | 213 (92.2%) | 105 (84.0%) | 60 (95.2%) |
Surgical resection | 289 (49.7%) | 74 (45.7%) | 148 (64.1%) | 49 (39.2%) | 18 (28.6%) |
Radiotherapy | 56 (9.6%) | 21 (13.0%) | 18 (7.8%) | 13 (10.4%) | 4 (6.3%) |
Response | |||||
Complete response | 360 (62.0%) | 94 (58.0%) | 164 (71.0%) | 71 (57.0%) | 31 (49.0%) |
Partial response | 62 (10.7%) | 16 (9.9%) | 16 (6.9%) | 19 (15.0%) | 11 (18.0%) |
Outcome | |||||
Relapse or Progression | 199 (34.3%) | 57 (35.2%) | 65 (28.1%) | 50 (40.0%) | 27 (42.9%) |
Dead | 152 (26.2%) | 44 (27.2%) | 47 (20.3%) | 36 (28.8%) | 25 (39.7%) |
Survival | |||||
Median OS | Not reached | Not reached | Not reached | 140 months | 61 months |
5-year OS | 67% | 65% | 72% | 67% | 55% |
Median PFS | 88 months | 55 months | 115 months | 66 months | 28 months |
5-year PFS | 53% | 50% | 62% | 50% | 37% |
Characteristics | DLBCL No. (%) | MALT No. (%) | BL No. (%) | MCL No. (%) | FL No. (%) | PTCL-U No. (%) | EATL No. (%) | ENKTL No. (%) |
---|---|---|---|---|---|---|---|---|
Treatment* | ||||||||
Chemotherapy | 368 (95.3) | 30 (49.2) | 29 (93.5) | 19 (100.0) | 5 (71.4) | 30 (88.2) | 23 (92.0) | 17 (94.4) |
Surgical resection | 223 (57.8) | 25 (41.0) | 9 (29.0) | 1 (5.3) | 3 (42.9) | 9 (26.5) | 12 (48.0) | 7 (38.9) |
Radiotherapy | 32 (8.3) | 13 (21.3) | 2 (6.5) | 1 (5.3) | 2 (28.6) | 4 (11.8) | 1 (4.0) | 1 (5.6) |
Response | ||||||||
Complete response | 264 (68.4) | 36 (59.0) | 22 (71.0) | 11 (57.9) | 4 (57.1) | 11 (32.4) | 7 (28.0) | 5 (27.8) |
Partial response | 36 (9.3) | 5 (8.2) | 5 (16.1) | 5 (26.3) | 0 (0.0) | 4 (11.8) | 4 (16.0) | 3 (16.7) |
Outcome | ||||||||
Relapse or Progression | 112 (29.0) | 14 (23.0) | 11 (35.5) | 8 (42.1) | 4 (57.1) | 19 (55.9) | 18 (72.0) | 13 (73.2) |
Dead | 87 (22.5) | 8 (13.1) | 7 (22.6) | 6 (31.6) | 2 (28.6) | 18 (52.9) | 15 (60.0) | 9 (50.0) |
Survival | ||||||||
Median OS | Not reached | Not reached | Not reached | 46 months | 54 months | 35 months | 8.6 months | 7 months |
5-year OS | 72% | 88% | 76% | 39% | 42% | 23% | 35% | 45% |
Median PFS | Not reached | 115 months | Not reached | 31 months | 16 months | 10 months | 4.2 months | 4 months |
5-year PFS | 58% | 80% | 60% | 0% | 22% | 17% | 23% | 21% |
Survival and prognostic factors
Characteristics | P value | Hazard ratio | 95% Confidence Interval | |
---|---|---|---|---|
Lower limit | Upper limit | |||
Age > 60 | < 0.001 | 1.945 | 1.379 | 2.743 |
Performance status ≥ 2 | < 0.001 | 2.072 | 1.384 | 3.101 |
Elevated serum LDH | 0.002 | 1.776 | 1.233 | 2.558 |
Extranodal involvement ≥ 2 | 0.579 | 0.892 | 0.596 | 1.335 |
Lugano stage IV | 0.001 | 1.248 | 1.090 | 1.429 |
Multiple intestinal involvement | 0.357 | 1.076 | 0.920 | 1.259 |
Immunophenotype T-cell | < 0.001 | 3.645 | 2.454 | 5.416 |
B symptoms | 0.028 | 1.530 | 1.046 | 2.237 |
Surgical resection not done | 0.281 | 1.235 | 0.842 | 1.811 |
Discussion
References | Study type | Time period | Nationality | number | Location | M/F | B/T cell | Stage I/II vs. III/IV | B-cell | T-cell |
---|---|---|---|---|---|---|---|---|---|---|
d'Amore et al [1] | Retrospective | 1983-1991 | Denmark | 109 | SI/LI | 76/33 | 93/16 | 56 vs. 48 | High grade (51) | PTCL (10) |
Intermediate grade (18) | ALCL (6) | |||||||||
Unknown (3) | Low grade (21) | |||||||||
Koch et al [4] | Retrospective | 1992-1996 | Germany | 58 | SI/LI | 40/18 | 48/10 | 52 vs. 6 | High grade (39) | T-cell (10) |
Low grade (4) | ||||||||||
BL/LBL (5) | ||||||||||
Kohno et al [6] | Retrospective | 1981-2000 | Japan | 143 | SI/LI | 109/34 | 122/21 | Not described | Large cell (84), BL (16) | PTCL (15) |
MALT (10), MCL (7) | ENKTL (2) | |||||||||
FL (4) | ALCL (2) | |||||||||
Daum et al [16] | Prospective | 1995-1999 | Germany | 56 | SI/LI | 25/31 | 21/35 | 42 vs. 14 | DLBCL (18) | EATL (28) |
MALT (2), FL (1) | Unknown (7) | |||||||||
Yin et al [12] | Retrospective | 1996-2005 | China | 34 | SI | 22/12 | 27/7 | 22 vs. 12 | DLBCL (24) | Unknown (7) |
MALT (3) | ||||||||||
Kako et al [10] | Retrospective | 1990-2007 | Japan | 23 | SI | 16/7 | 20/3 | 11 vs. 12 | DLBCL (15), FL (1) | EATL (2) |
MCL (1), MALT (2) | ALCL (1) | |||||||||
Unknown (1) | ||||||||||
Li et al [9] | Retrospective | 1992-2003 | China | 40 | SI/LI | 26/14 | 38/2 | 28 vs. 12 | DLBCL (17) | PTCL (1) |
MALT (20) | Unknown (1) | |||||||||
Unknown (1) | ||||||||||
Wong et al [8] | Retrospective | 1989-1999 | Singapore | 14 | LI | 13/1 | 14/0 | 5 vs. 9 | DLBCL (8), MCL (4) | |
BL (2) |