Table 1.
Case | Age/sex | Tumor | Site/size (cm)/MF | Histotype | Risk category | Other diseases | Mutation status | Follow-up | |
---|---|---|---|---|---|---|---|---|---|
Outcome | Months | ||||||||
1 | 37/F | 1a 1b |
Stomach/9/>5 Stomach/4/>5 |
S S |
HR IR |
Pulmonary chondromas | WT WT |
NED | 113 |
2 | 39/F | 2a 2b 2c |
Stomach /11/>5 Stomach/3.5/>5 Small intestine/too small/>5 |
M M M |
HR IR IR |
Adrenal adenoma Pulmonary chondromas |
WT WT NA |
NED | 111 |
3 | 46/F | 3a 3b |
Duodenum/2.0/<5 Stomach/0.5/<5 |
S S |
VLR VLR |
Absent | NA NA |
NED | 122 |
4 | 51/M | 4a 4b |
Stomach/4.0/<5 Stomach/1.0/<5 |
S S |
LR VLR |
Absent | KIT exon11: c.1728_1739dup NA |
NED | 57 |
5 | 51/F | 5a 5b |
Small intestine/4.5/>10 Small intestine/0.5/>10 |
S S |
HR HR |
Breast cancer | WT WT |
NED | 183 |
6 | 52/M | 6a 6b 6c |
Small intestine/13/>5 Small intestine/4/>5 Small intestine/2/>5 |
S S S |
HR HR IR |
Absent | KIT exon11:c.1655_1666del WT NA |
AWD | 62 |
7 | 55/F | 7a | Stomach/6.0/<5 | S | IR | Neurofibromas | WT | NED | 37 |
7b | Small intestine/2/<5 | S | VLR | NA | |||||
7c | Small intestine/1/<5 | S | VLR | NA | |||||
7d | Small intestine/1/<5 | S | VLR | NA | |||||
7e | Small intestine/0.5/<5 | S | VLR | NA | |||||
7f | Small intestine/0.5/<5 | S | VLR | NA | |||||
8 | 57/M | 8a 8b 8c |
Mesentery/17/>5 Mesentery/11/>5 Mesentery/2/>5 |
S S S |
HR HR IR |
Absent | KIT exon11: p.M552_Q556del NA NA |
DOD | 40 |
9 | 58/F | 9a 9b |
Stomach/2/<5 Stomach/0.9/<5 |
S S |
LR VLR |
Breast cancer | KIT exon11: Q556-E561 Del KIT exon11:Ins QPS+V560E |
NED | 3 |
10 | 59/F | 10a 10b 10c |
Rectum/3/>5 Rectum/1/>5 Rectum/too small/>5 |
S S S |
HR IR IR |
Absent | PDGFRA exon12: p.P567P WT WT |
NED | 158 |
11 | 64/F | 11a 11b 11c 11d 11e 11f |
Small intestine/6/<5 Small intestine/4.5/<5 Small intestine/2/<5 Small intestine/2/<5 Small intestine/2/<5 Small intestine/1.8/<5 |
S S S S S S |
HR LR VLR VLR VLR VLR |
Neurofibromas | PDGFRA exon18:p.V824V PDGFRA exon18:p.V824V PDGFRA exon18:p.V824V PDGFRA exon18:p.V824V PDGFRA exon18:p.V824V NA |
NED | 125 |
12 | 66/M | 12a 12b |
Stomach/3/<5 Stomach/0.4/<5 |
S S |
LR VLR |
Absent | WT NA |
NED | 65 |
13 | 68/M | 13a 13b 13c |
Small intestine/9/<5 Small intestine/3.8/<5 Small intestine/1.9/<5 |
S S S |
HR LR VLR |
Absent | NA NA NA |
DOPC | / |
14 | 69/M | 14a 14b |
Stomach/2.9/<5 Stomach/1.5/<5 |
S S |
LR VLR |
Absent | PDGFRA exon 18 : p.V824V KIT exon11: p.Y578_D579dup |
DUNC | 47 |
15 | 72/M | 15a 15b |
Stomach/5/<5 Stomach/1/<5 |
M M |
LR VLR |
Absent | PDGFRA exon 12: p.P567P NA |
DOD | 24 |
16 | 73/F | 16a 16b |
Stomach/3.5/>10 Stomach/2.5/>10 |
M M |
HR HR |
Absent | KIT exon11: p.T574_R588dup PDGFRA exon18: p.D842V |
DUNC | 52 |
17 | 74/F | 17a 17b |
Stomach/1.6/<5 Stomach/0.3/<5 |
S S |
LR VLR |
Absent | NA NA |
NED | 100 |
18 | 76/F | 18a 18b 18c |
Stomach/8/>5 Stomach/2/>5 Stomach/too small/>5 |
S S S |
HR IR IR |
Absent | KIT exon11: p.V559_T574del WT WT |
NED | 142 |
19 | 79/M | 19a | Small intestine/6.5/>5 | S | HR | Absent | KIT exon11: p.Y570_L576del | DOD | 118 |
19b | Small intestine/1.2/>5 | S | IR | KIT exon11: p.Y570_L576del | |||||
20 | 80/M | 20a 20b |
Stomach/3.5/<5 Stomach/1.5/<5 |
E S |
IR VLR |
Absent | KIT exon11: p.W557R PDGFRA exon18: p.D842V |
NED | 66 |
Abbreviations: AWD, alive with disease; NED, no evidence of disease; DOD, dead of disease; DOPC, dead of postoperative complications; DUNC, dead of unknown causes; E, epithelioid; S, spindle; MF, mitotic figures, (50/HPF); VLR, very low risk; LR, low risk; IR, intermediate risk; HR, high risk; WT, wild-type; NA, not available.