Table 1.
Clinical characteristics of 45 cases of Epithelioid Inflammatory Myofibroblastic Sarcoma.
| Case | Author | Age | Sex | Size (cm); Greatest dimension | Site | Treatment | Follow up/status | Fusion gene |
|---|---|---|---|---|---|---|---|---|
| 1-11 | Enrıquez 7 | 59y | M | 15 | Mesentery of the small bowel | Surgery + CT | 12m/DOD | RANBP2-ALK in 3 cases |
| 41y | M | 26 | Omentum | Surgery + CT +ALKI | 40m/alive | |||
| 6y | M | 10.5 | Omentum | Surgery + CT | 13m/alive | |||
| 28y | M | NA | Mesentery of the small bowel | NA | NA | |||
| 63y | M | 25 | Mesentery of the small bowel | Surgery + CT | 3m/DOD | |||
| 42y | M | NA | Intra-abdominal | Surgery + CT | 13m/alive | |||
| 7m | M | 10 | Peritoneum | Surgery + CT +RT | 36m/DOD | |||
| 40y | M | 8 | Peritoneum | Surgery + CT +RT | 28m/DOD | |||
| 31y | F | 17.5 | Mesentery of the small bowel | Surgery + CT | 11m/DOD | |||
| 6y | M | 14 | Omentum and mesentery | Surgery | NA | |||
| 39y | M | 15 | Mesentery of the small bowel | Surgery | NA | |||
| 12 | Batool et al 9 | 4m | F | 12 | Mesentery of the small bowel | Surgery | 6m | NA |
| 13 | Halllin 10 | NA | M | NA | Intra-abdominal | NA | NA | NA |
| 14 | Du et al 1 | 26y | M | 25 | Intra-abdominal + abdominal wall | Surgery +CT | DOD | RANBP2-ALK |
| 15 | Xu et al 11 | 35y | F | 4 | Stomach | Surgery | 10m/alive | NA |
| 16 | Garg et al 12 | 2y | F | NA | Omentum+mesentry | Surgery | 7m / alive | NA |
| 17 | Xu et al 13 | 28y | M | NA | Intra-abdominal + abdominal wall | ALKI | 12m/alive | RANBP2-ALK/chromosomal ALK-G1269A mutation |
| 18 | Fu et al 14 | 21y | M | 10 | Lung | Surgery + ALKI | 4m/DOD | NA |
| 19 | Liu et al 15 | 22y | M | 13 | Intra-abdominal | Surgery + ALKI | 16m/alive | RANBP2-ALK |
| 20 | Wan et al 16 | 14y | M | 18 | Retroperitoneal | Surgery | 3m/DOD | NA |
| 21-25 | Yu et al 8 | 37y | F | 5 | Rectum | Surgery | 8m/alive | NA |
| 55y | M | NA | Terminal ileum | Surgery +CT | 10m/alive | |||
| 22y | M | 20 | Mesentery of colon | Surgery + ALKI | 14m/alive | |||
| 58y | F | NA | Omentum | Surgery +CT | 8m/DOD | |||
| 15y | F | 12 | Transverse colon | Surgery | 7m/alive | |||
| 26 | Liu et al 17 | NA | F | 10.9 | Sigmoid | Surgery + ALKI | NA | RANBP2-ALK |
| 27 | Wu et al 18 | 47y | F | 7.5 | Mesentery of colon | Surgery +CT | 4m/DOD | RANBP2-ALK |
| 28 | Zhang and Wang 19 | 46y | F | 11 | Intra-abdominal | Surgery + ALKI | DOD | NA |
| 29 | Singh et al 20 | 25y | M | 6.9 | Lung | Surgery + ALKI | 4m/alive | NA |
| 30 | Azad et al 21 | 53y | F | NA | Intrapericardial | Surgery +CT | DOD | No fusion genes |
| 31 | Collins et al 22 | 43y | F | NA | Uterine | Surgery * NA | 22m/alive | RANBP2-ALK |
| 32 | Fang et al 23 | 15y | F | NA | Ovary | Surgery + CT +ALKI | 2y/alive | RANBP2-ALK |
| 33 | Sarmiento et al 24 | 71y | M | 12.5 | Intrapleural | Surgery +ALKI | 1y/alive | NA |
| 34 | Kozu et al 25 | 57y | M | NA | Pleural cavity or chest wall | ALKI | NA | RANBP2-ALK |
| 35 | Kimbara et al 26 | 22y | M | 6 | Intra-abdominal | Surgery + CT +ALKI | 10m/alive | RANBP2-ALK |
| 36 | Wang et al 27 | 42y | F | 19 | Greater omentum | Surgery +ALKI | alive | PRRC2B-ALK |
| 37 | Zhou et al 28 | 8y | M | 18 | Intra-abdominal | Surgery | DOD | NA |
| 38 | Bai et al 29 | 65y | M | 9.3 | Descending colon | Surgery | 18m | NA |
| 39-43 | Lee et al 30 | 34y | M | 8 | Liver | Surgery | DOD | RANBP2-ALK in all cases |
| 62y | M | 25 | Omentum | Surgery+CT | DOD | |||
| 76y | F | 9 | Small bowel serosa | Surgery | DOD | |||
| 30y | M | 10 | Intra-abdominal | Surgery | DOD | |||
| 16y | F | 8 | Lung | Surgery+CT+RT+ALKI | alive | |||
| 44 | Chopra et al 31 | 72y | F | 4.7 | CNS | Surgery +ALKI | alive | VCL–ALK |
| 45 | Aminimoghaddam Current case | 20y | F | 21 | Peritoneum | Surgery+CT+ ALKI | DOD | NA |
Abbreviations: ALKI, ALK inhibitor; cm, centimeter,; CNS, central nervous system; CT, chemotherapy; DOD, dead of disease; F, female; M, male,; m, month; NA, not available; RT, radiotherapy; y, year.