Table 1.

Patients' baseline characteristics.

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)