Characteristics | Female | Male | Overall | |
---|---|---|---|---|
(N = 31) | (N = 74) | (N = 105) | ||
Histological report years, n (%) | Â | Â | Â | Â |
 < 1981 |  | 2 (6%) | 17 (23%) | 19 (18.1%) |
 1981–1990 |  | 2 (6%) | 10 (14%) | 12 (11.4%) |
 1991–2000 |  | 7 (23%) | 14 (19%) | 21 (20.0%) |
 2001–2009 |  | 7 (23%) | 13 (18%) | 20 (19.0%) |
 > 2009 |  | 11 (35%) | 20 (27%) | 31 (29.5%) |
 Not Available |  | 2 (6%) | 0 (0%) | 2 (1.9%) |
SPC* age onset, year | Median (IQR) | 37 (30, 50) | 33.5 (25, 43) | 34 (26.5, 44) |
Histological grade, n (%) | Â | Â | Â | Â |
 1 |  | 22 (71%) | 37 (50%) | 59 (56.2%) |
 2 |  | 7 (23%) | 33 (45%) | 40 (38.1%) |
 3 |  | 1 (3%) | 3 (4%) | 4 (3.8%) |
 Not Available |  | 1 (3%) | 1 (1%) | 2 (1.9%) |
Axial site, n (%) | Â | Â | Â | Â |
 No |  | 12 (39%) | 33 (45%) | 45 (42.9%) |
 Yes |  | 19 (61%) | 41 (55%) | 60 (57.1%) |
Cartilage cap thickness, cm | Median (IQR) | 3 (2.5, 5.5) | 3 (2, 5.75) | 3 (2.2, 5.5) |
 Diameter, cm | Median (IQR) | 10.5 (8, 15) | 11 (8.5, 14.3) | 11 (8.5, 14.6) |
Surgical treatment, n (%) | Â | Â | Â | Â |
 Amputation |  | 5 (16%) | 10 (14%) | 15 (14.3%) |
 Complete resection |  | 14 (45%) | 30 (41%) | 44 (41.9%) |
 Debulking |  | 8 (26%) | 21 (28%) | 29 (27.6%) |
 Partial resection |  | 1 (3%) | 8 (11%) | 9 (8.6%) |
 Not Available |  | 3 (10%) | 5 (7%) | 8 (7.6%) |
Recurrence/Metastasis, n (%) | Â | Â | Â | Â |
 No |  | 27 (87%) | 56 (76%) | 83 (79.0%) |
 Yes |  | 4 (13%) | 18 (24%) | 22 (21.0%) |
Death cause, n (%) | Â | Â | Â | Â |
 Alive |  | 31 (100%) | 66 (89%) | 97 (92.4%) |
 Dead for disease |  | 0 (0%) | 3 (4%) | 3 (2.9%) |
 Dead for other causes |  | 0 (0%) | 5 (7%) | 5 (4.8%) |
Germinal variant, n (%) | Â | Â | Â | Â |
 No |  | 1 (3%) | 2 (3%) | 3 (2.9%) |
 Yes |  | 13 (42%) | 34 (46%) | 47 (44.8%) |
 Not Available |  | 17 (55%) | 38 (51%) | 55 (52.4%) |
Gene, n (%) | Â | Â | Â | Â |
 EXT1 |  | 10 (77%) | 21 (62%) | 31 (66%) |
 EXT2 |  | 3 (23%) | 13 (38%) | 16 (34%) |
Variant type, n (%) | Â | Â | Â | Â |
 Big Rearrangement |  | 0 (0%) | 4 (12%) | 4 (9%) |
 Deletion |  | 1 (8%) | 1 (3%) | 2 (4%) |
 Duplication |  | 1 (8%) | 2 (6%) | 3 (6%) |
 Frameshift |  | 4 (31%) | 14 (41%) | 18 (38%) |
 Missense |  | 3 (23%) | 1 (3%) | 4 (9%) |
 Nonsense |  | 3 (23%) | 10 (29%) | 13 (28%) |
 Splicesite |  | 1 (8%) | 2 (6%) | 3 (6%) |