Clinical features of multiple gastrointestinal stromal tumors: A pooling analysis combined with evidence and gap map

Table 1 Baseline characteristics of patients with multiple gastrointestinal stromal tumors, n (%)

	PKUPH patients, n = 121	Literature-based patients, n = 1611	Total patients, n = 1731
Sex	n = 12	n = 160	n = 172
Female	6 (50.00)	96 (60.00)	102 (59.30)
F/M	1.00	1.48	1.46
Age in yr	n = 12	n = 161	n = 173
Range	53-88	8-84	8-88
≤ 20	0 (0.00)	25 (15.63)	25 (14.45)
21-40	0 (0.00)	29 (18.12)	29 (16.86)
41-60	2 (16.67)	51 (31.88)	53 (30.81)
61-80	9 (75.00)	51 (31.88)	60 (34.88)
> 80	1 (8.33)	5 (3.13)	6 (3.49)
Mean (SD)	65.33 (9.48)	48.70 (21.17)	49.85 (20.99)
Symptoms	n = 12	n = 55	n = 67
GI bleeding	2 (16.67)	31 (56.36)	33 (49.25)
Hematochezia	1 (8.33)	13 (24.07)	14 (21.21)
Anemia	0 (0.00)	11 (20.37)	11 (16.67)
Hematemesis	1 (8.33)	2 (3.70)	3 (4.55)
Incidental finding	7 (58.33)	15 (27.27)	22 (32.83)
Abdominal pain	2 (16.67)	14 (25.45)	16 (23.88)
Palpable mass	0 (0.00)	2 (3.64)	2 (2.99)
Others	1 (8.33)	11 (20.00)	12 (17.91)
Classification	n = 12	n = 161	n = 173
Sporadic multiple GIST	10 (83.33)	53 (32.92)	63 (36.42)
NF-1 associated GIST	2 (16.67)	72 (44.72)	74 (42.77)
Primary familial GIST	0 (0.00)	8 (4.97)	8 (4.62)
Pediatric GIST	0 (0.00)	25 (15.53)	25 (14.45)
Carney-Stratakis syndrome	0 (0.00)	0 (0.00)	0 (0.00)
Carney triads	0 (0.00)	3 (1.86)	3 (1.73)
Combined diseases	n = 12	n = 161	n = 173
GI tumors	0 (0.00)	3 (1.86)	3 (1.73)
GU tumors	0 (0.00)	5 (3.11)	5 (2.89)
Breast tumors	1 (8.33)	7 (4.35)	8 (4.62)
Other tumors	0 (0.00)	5 (3.11)	5 (2.89)

¹n For total number of patients, other n for number of patients with relevant data. GI: Gastrointestinal; GIST: Gastrointestinal stromal tumor; GU: Genitourinary tract; PKUPH: Peking University People’s Hospital; SD: Standard deviation.

Table 2 Tumor and pathological features of patients with multiple gastrointestinal stromal tumors, n (%)

	PKUPH patients, n = 121	Literature-based patients, n = 1611	Total patients, n = 1731
Site	n = 40	n = 330	n = 370
Stomach	22 (55.00)	121 (36.67)	143 (38.65)
Small intestine	17 (42.50)	201 (60.91)	218 (58.92)
Duodenum	1 (2.50)	27 (8.18)	28 (7.57)
Jejunum	4 (10.00)	68 (20.61)	72 (19.46)
Ileum	3 (7.50)	25 (7.58)	28 (7.57)
Other sites	1 (2.50)	8 (2.42)	9 (2.43)
Involving organ	n = 12	n = 156	n = 168
Single	9 (75.00)	108 (69.23)	117 (69.64)
Two or more	3 (25.00)	48 (30.77)	51 (30.36)
Size in cm	n = 40	n = 315	n = 355
Range	0.10-8.00	0.05-27.00	0.05-27.00
≤ 1	22 (55.00)	94 (29.84)	116 (32.68)
1-2	4 (10.00)	65 (20.64)	69 (19.44)
2-5	11 (27.75)	98 (31.11)	109 (30.70)
5-10	3 (7.50)	45 (14.29)	48 (13.52)
> 10	0 (0.00)	13 (4.13)	13 (3.56)
Mean (SD)	2.00 (2.16)	3.26 (3.50)	3.12 (3.40)
Cellular type	n = 40	n = 180	n = 220
Spindle	40 (100.00)	137 (76.11)	177 (80.46)
Epithelial	0 (0.00)	23 (12.78)	23 (10.46)
Mixed	0 (0.00)	20 (11.11)	20 (9.09)
Mitosis, /50 HPFs	n = 35	n = 202	n = 237
Range	0-9	0-48	0-48
≤ 5	33 (94.29)	174 (86.14)	207 (87.34)
5-10	2 (5.71)	15 (7.43)	17 (7.17)
> 10	0 (0.00)	13 (6.44)	13 (5.49)
Mean (SD)	3.86 (1.87)	3.32 (5.91)	3.40 (5.50)
Risk classification	n = 35	n = 249	n = 284
Very low risk	20 (57.14)	54 (21.69)	74 (26.05)
Low risk	10 (28.57)	113 (45.38)	123 (43.31)
Median risk	3 (8.57)	26 (10.44)	29 (10.21)
High risk	2 (5.71)	56 (22.49)	58 (20.42)
CD117	n = 35	n = 149	n = 184
Positive	35 (100.00)	144 (96.64)	179 (97.28)
CD34	n = 35	n = 140	n = 175
Positive	12 (34.29)	114 (81.43)	126 (72.00)
Ki-67	n = 35	n = 47	n = 82
Range	0-20	1-33.8	0-33.8
Mean (SD)	3.83 (4.85)	3.96 (5.11)	3.91 (4.97)
Desmin	n = 35	n = 98	n = 133
Positive	1 (2.86)	1 (1.02)	2 (1.50)
S-100	n = 35	n = 108	n = 143
Positive	1 (2.86)	32 (29.63)	33 (23.08)
SMA	n = 35	n = 120	n = 155
Positive	5 (14.29)	19 (15.83)	24 (15.48)

¹n For total number of patients, other n for number of patients with relevant data. HPFs: High-power fields; PKUPH: Peking University People’s Hospital; SD: Standard deviation.

Table 3 Perioperative information and follow-up results of patients with multiple gastrointestinal stromal tumors, n (%)

	PKUPH patients, n = 121	Literature-based patients, n = 1611	Total patients, n = 1731
Preoperative I.E.	n = 12	n = 29	n = 41
CT	12 (100.00)	19 (65.52)	31 (75.61)
MRI	2 (16.67)	2 (6.90)	4 (9.76)
Endoscopy	6 (50.00)	21 (72.41)	27 (65.85)
Approach	n = 12	n = 25	n = 37
Laparotomy	5 (41.67)	18 (72.00)	23 (62.16)
Laparoscopy	7 (58.33)	7 (28.00)	14 (37.84)
Excision Extension	n = 12	n = 30	n = 42
Radical resection	9 (75.00)	22 (73.33)	31 (73.81)
Palliative resection	3 (25.00)	8 (26.67)	11 (26.19)
Imatinib	n = 12	n = 37	n = 49
Apply	7 (58.33)	25 (67.57)	32 (65.31)
Not apply	5 (41.67)	12 (32.43)	17 (34.70)
Follow-up time in mo	n = 12	n = 114	n = 126
Range	3-86	3-396	3-396
Mean (SD)	33.75 (27.28)	83.01 (82.26)	78.32 (79.96)
Outcome	n = 12	n = 123	n = 135
ANED	12 (100.00)	77 (62.60)	89 (65.93)
AWD	0 (0.00)	21 (17.07)	21 (15.56)
ATSU	0 (0.00)	2 (1.63)	2 (1.48)
DOD	0 (0.00)	7 (5.69)	7 (5.19)
DUC	0 (0.00)	9 (7.32)	9 (6.67)
DOPC	0 (0.00)	2 (1.63)	2 (1.48)
DUNC	0 (0.00)	5 (4.07)	5 (3.70)
Recurrence	0/12 (0.00)	3/123 (2.44)	3/135 (2.22)
Metastasis	n = 12	n = 123	n = 135
Lymph node	0 (0.00)	5 (4.07)	5 (3.70)
Liver	0 (0.00)	8 (6.50)	8 (5.93)
Peritoneum	0 (0.00)	9 (7.32)	9 (6.67)
Lung	0 (0.00)	1 (0.76)	1 (0.74)
Abdomen	0 (0.00)	1 (0.76)	1 (0.74)
Omentum	0 (0.00)	1 (0.76)	1 (0.74)

¹n For total number of patients, other n for number of patients with relevant data. CT: Computed tomography; I.E.: Imaging examinations; MRI: Magnetic resonance imaging; PKUPH: Peking University People’s Hospital; SD: Standard deviation.