Table 1.
Clinical characteristics of mesenchymal tumors with DICER1 alteration according to LGMT DICER1, SARC DICER1 and PIS DICER1 cluster assignment (n = 86)
| Molecular class | LGMT DICER1 | SARC DICER1 | PIS DICER1 | |
|---|---|---|---|---|
| Number of patients | n = 86 | 20 | 38 | 28 |
| Diagnoses | PIS DICER1 | 0 (0%) | 0 (0%) | 26 (100%) |
| NCMH | 2 (100%) | 0 (0%) | 0 (0%) | |
| PB | 0 (0%) | 2 (100%) | 0 (0%) | |
| PPB I/Ir | 6 (100%) | 0 (0%) | 0 (0%) | |
| PPBII | 3 (50%) | 3 (50%) | 0 (0%) | |
| PPB III | 0 (0%) | 8 (89%) | 1 (11%) | |
| CN | 7 (100%) | 0 (0%) | 0 (0%) | |
| AS | 0 (0%) | 3 (75%) | 1 (25%) | |
| ERMS | 2 (8%) | 22 (92%) | 0 (0%) | |
| Location | CNS | 0 (0%) | 0 (0%) | 26 (100%) |
| Head and Neck | 2 (67%) | 1 (33%) | 0 (0%) | |
| Lungs | 9 (39%) | 13 (57%) | 1 (4%) | |
| Kidney | 7 (64%) | 3 (27%) | 1 (9%) | |
| Retroperitoneal | 0 (0%) | 1 (100%) | 0 (0%) | |
| Fallopian tube | 1 (100%) | 0 (0%) | 0 (0%) | |
| Uterus | 1 (5%) | 20 (95%) | 0 (0%) | |
| Outcome | 5 year DSS | 100% | 74.1% (CI 52.6−100) | 56.6% (CI 35.0−91.4) |
| 5 year PFS | 90.9% (CI 75.4−100) | 47.5% (CI 26.5−85.4) | 26.2% (CI 6.1−100) | |
| Age | Range (years) | 0.1−18 | 2.0−69.0 | 0.8−61 |
| Median (years) | 1.5 | 23 | 6 | |
| NA | 1 | 1 | 0 | |
| Gender | Female | 9 | 29 | 15 |
| Male | 11 | 9 | 13 | |
| Ratio (M:F) | 1.2 | 0.3 | 0.9 |
DSS Disease specific survival, PFS Progression free survival.