Table 3. Characteristics of the ISKS cohort.
| Probands | ||
| TP53 | non-TP53 | |
| n | 17 | 539 |
| Gender | ||
| Male | 9 (53%) | 291 (54%) |
| Female | 8 (47%) | 248 (46%) |
| Median age at diagnosis (yrs±SD) | ||
| First cancer | 33±14 | 48±18 |
| Range | (10–59) | (3–93) |
| Sarcoma | 36±17 | 50±18 |
| Range | (16–68) | (3–93) |
| Individuals with multiple primaries | 8 (47%) | 83 (15%) |
| Number of tumours/individual (mean±SD) | 2.5±0.5 | 2.2±0.5 |
| Sarcoma subtypes | ||
| n | 19* | 546* |
| Bone | ||
| Osteosarcoma | 3 (16%) | 52 (10%) |
| Chondrosarcoma | 2 (11%) | 48 (9%) |
| Ewing/Primitive neuroectodermal tumour | 1 (5%) | 47 (9%) |
| Other | – | 3 (1%) |
| Soft tissue | ||
| Undifferentiated pleomorphic sarcoma | 3 (16%) | 67 (12%) |
| Leiomyosarcoma | 4 (21%) | 61 (11%) |
| Fibromyxosarcoma | – | 51 (9%) |
| Well differentiated/Dedifferentiate LPS | 1 (5%) | 43 (8%) |
| Myxoid LPS | – | 27 (5%) |
| LPS not otherwise specified | 2 (11%) | 14 (3%) |
| Synovial sarcoma | – | 38 (7%) |
| Angiosarcoma | 1 (5%) | 8 (1%) |
| Epithelioid sarcoma | – | 8 (1%) |
| Malignant peripheral nerve sheath tumour | – | 8 (1%) |
| Rhabdomyosarcoma | 1 (5%) | 7 (1%) |
| Desmoplastic small round cell tumour | 1 (5%) | – |
| Other | – | 64 (12%) |
| Family history | ||
| Classic Li Fraumeni Syndrome | 4 (24%) | 4 (<1%) |
| Chompret Li Fraumeni Like | 6 (35%) | 44 (8%) |
| Other familial cancer syndrome | – | 8 (1%) |
| No family history | 7 (41%) | 458 (85%) |
| Uninformative | – | 25 (5%) |
*
some probands have >1 sarcoma; SD, standard deviation; LPS, liposarcoma.