TABLE 1.
Patient Characteristics
| Histologic subtype | LGFMS | SEF | H-LGFMS/SEF |
|---|---|---|---|
| Number of pts with primary disease (%) | 239/294 (81.3) | 32/294 (10.9) | 23/294 (7.8) |
| Age at surgery (y), median (IQR) | 38.0 (27.5-52.0) | 47.0 (31.8-55.0) | 45.0 (30.0-58.0) |
| Male/female (%) | 119 (49.8)/120 (50.2) | 23 (71.9)/9 (28.1) | 11 (47.8)/12 (52.2) |
| Histopathologic/molecular features | |||
| MUC4 expression (%) | |||
| Positive | 170 (71.1) | 24 (75.0) | 19 (82.6) |
| Negative | 3 (1.3) | 0 (0.0) | 0 (0.0) |
| Unknown | 66 (27.6) | 8 (25.0) | 4 (17.4) |
| Number of pts analyzed for FUS rearrangement (%) | 158/239 (66.1) | 2/32 (6.3) | 16/23 (73.9) |
| Positive | 152/239 (63.6) | 2/32 (6.3) | 8/23 (34.8) |
| FUS::CREB3L2 | 77/152 (50.7) | 1/2 (50.0) | 3/9 (33.3) |
| FUS::CREB3L1 | 6/152 (3.9) | 0/2 (0.0) | 0/9 (0.0) |
| No fusion partner specified | 69/152 (45.4) | 1/2 (50.0) | 5/9 (55.6) |
| Negative | 6/239 (2.5) | 0/32 (0.0) | 8/23 (34.8) |
| Number of pts analyzed for EWSR1 rearrangement (%) | 2/239 (0.8) | 17/32 (53.1) | 9/23 (39.1) |
| Positive | 2/239 (0.8) | 15/32 (46.8) | 5/23 (21.7) |
| EWSR1::CREB3L1 | 2/2 (100.0) | 11/15 (73.3) | 4/5 (80.0) |
| EWSR1-CREB3L2 | 0/2 (0.0) | 2/15 (13.3) | 0/5 (0.0) |
| No fusion partner specified | 0/2 (0.0) | 2/15 (13.3) | 1/5 (20.0) |
| Negative | 0/239 (0.0) | 2/32 (6.3) | 4/23 (17.4) |
| Primary site (%) | |||
| Extremities | 153 (64.0) | 14 (43.8) | 11 (47.8) |
| Abdomen / retroperitoneum | 21 (8.8) | 4 (12.5) | 5 (21.7) |
| Trunk | 33 (13.8) | 4 (12.5) | 3 (13.0) |
| Other | 32 (13.4) | 10 (31.3) | 4 (17.4) |
| Treatments of primary disease | |||
| Surgery (%) | |||
| No | 0 (0.0) | 0 (0.0) | 0 (0.0) |
| Yes | 239 (100.0) | 32 (100.0) | 23 (100.0) |
| R0 | 195 (81.6) | 28 (87.5) | 19 (82.6) |
| R1 | 28 (11.7) | 1 (3.1) | 2 (8,7) |
| Missing | 13 (5.4) | 1 (3.1) | 1 (4.3) |
| R2 | 3 (1.3) | 2 (6.3) | 1 (4.3) |
| Radiotherapy (%) | |||
| No | 171 (74.1) | 16 (50.0) | 14 (60.9) |
| Yes | 62 (25.9) | 16 (50.0) | 9 (39.1) |
| Systemic therapies (%) | |||
| No | 235 (98.3) | 24 (75.0) | 22 (95.7) |
| Yes | 4 (1.7) | 8 (25.0) | 1 (4.3) |
| Status at last follow-up (%) | |||
| Alive, No evidence of disease | 211 (88.3) | 19 (59.4) | 17 (73.9) |
| Alive, With evidence of disease | 5 (2.1) | 7 (21.9) | 2 (8.7) |
| Dead | 4 (1.7) | 6 (18.8) | 2 (8.7) |
| Lost to follow-up | 19 (7.9) | 0 (0.0) | 2 (8.7) |
pts indicates patients.