| | Characteristic | All (N = 25) | NF1 MPNST (N = 17) | Sporadic MPNST (N = 8) |
| | Median age at diagnosis, years (range) | 37 (19, 81) | 28 (19, 63) | 61 (19, 81) | | Female, n (%) | 15 (60) | 10 (58.8) | 5 (62.5) | | Asian, n (%) | 1 (4) | 1 (5.9) | 0 (0) | | Black or African American, n (%) | 8 (32) | 5 (29.4) | 3 (37.5) | | White, n (%) | 16 (64) | 11 (64.7) | 5 (62.5) | | Primary tumor location, n (%) | | | | | Extremities | 10 (40) | 7 (41.3) | 3 (37.5) | | Trunk | | | | | Chest wall | 3 (12) | 3 (17.6) | 0 (0) | | Spine | 2 (8) | 2 (11.8) | 0 (0) | | Mediastinal soft tissue | 1 (4) | 1 (5.9) | 0 (0) | | Pelvis | 2 (8) | 2 (11.8) | 0 (0) | | Retroperitoneum | 1 (4) | 0 (0) | 1 (12.5) | | Abdominal wall | 1 (4) | 1 (5.9) | 0 (0) | | Axilla | 2 (8) | 1 (5.9) | 1 (12.5) | | Parotid gland | 1 (4) | 0 (0) | 1 (12.5) | | Primary tumor resected, n (%) | 20 (80) | 13 (76.5) | 7 (87.5) | | Margins of surgical resection, n (%) | | | | | R0: microscopic negative | 9 (45) | 7 (53.8) | 2 (28.6) | | R1: microscopic positive | 4 (20) | 2 (15.4) | 2 (28.6) | | R2: gross residual disease | 5 (25) | 3 (23.1) | 2 (28.6) | | If resected, did the primary tumor locally recur, n (%) | N = 20 | N = 13 | N = 7 | | No | 5 (25) | 2 (15.4) | 3 (42.9) | | Yes | 15 (75) | 11 (84.6) | 4 (57.1) | | Metastatic disease at diagnosis, n (%) | N = 21 | N = 13 | N = 8 | | No | 18 (86) | 12 (92.3) | 6 (75) | | Yes | 3 (14) | 1 (7.7) | 2 (25) | | Metastatic disease at enrollment, n (%) | N = 21 | N = 13 | N = 8 | | No | 2 (10) | 1 (7.7) | 1 (12.5) | | Prior chemotherapy, n (%) | | | | | No | 2 (8) | 2 (11.8) | 0 (0) | | Yes | 23 (92) | 15 (88.2) | 8 (100) | | No. of prior treatment regimens | | | | | Median (range) | 2 (0, 5) | 2 (0, 5) | 2 (1, 4) | | Patient had prior radiation, N (%) | | | | | No | 4 (16) | 3 (17.6) | 1 (12.5) | | Yes | 21 (84) | 14 (82.4) | 7 (87.5) | | No. of prior radiation treatments | | | | | Median (range) | 1 (0, 3) | 1 (0, 3) | 1 (0, 2) | | Patient had prior surgeries, N (%) | | | | | No | 2 (8) | 1 (5.9) | 1 (12.5) | | Yes | 23 (92) | 16 (94.1) | 7 (87.5) | | No. of prior surgeries | | | | | Median (range) | 2 (0, 8) | 2 (0, 8) | 2 (0, 5) | | ECOG at baseline, N (%) | | | | | 0 | 12 (48) | 8 (47.1) | 4 (50) | | 1 | 11 (44) | 7 (41.2) | 4 (50) | | 2 | 2 (8) | 2 (11.8) | 0 (0) |
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