Table 1 Clinical characteristics of mesenchymal tumors with DICER1 alteration according to LGMT DICER1, SARC DICER1 and PIS DICER1 cluster assignment (n = 86)
From: Genomic characterization of DICER1-associated neoplasms uncovers molecular classes
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 |
