Table 1.
Demographics and outcome for all enrolled individuals (n=107) with ovarian sex cord- stromal tumors.
| N=107 | SLCT | JGCT | GAB | Sex Cord tumor with annular tubules | Sertoli cell tumor | Steroid cell tumor | AGCT | Sclerosing stromal tumor | Sex Cord tumor, NOS |
|---|---|---|---|---|---|---|---|---|---|
| Patients (n) | 49 (46% ) | 25 (23% ) | 5 (5%) | 5 (5%) | 5 (5%) | 2 (2%) | 10 (9%) | 4 (4%) | 2 (2%) |
| Caucasian (%) | 37 (76% ) | 10 (40% ) | 4 (80%) | 4 (80%) | 5 (100% ) | 0 | 6 (60% ) | 2 (50%) | 1 (50% ) |
| Age @ Diagnosis, Median (years), range | 17 (2– 61) | 9 (<1– 28) | 16 (14– 32) | 12 (3– 50) | 44 (33– 52) | 38 (17– 58) | 37 (13– 49) | 14 (10– 22) | 12 (10– 15) |
| Median Time from Diagnosis (months), range | 32 (0– 624) | 15 (0– 415) | 11 (3– 117) | 23 (0– 47) | 33 (18– 68) | 11 (1–– 20) | 46 (3– 203) | 35 (1–76) | 7.5 (1– 14) |
| Status | |||||||||
| NED | 40 (82%) | 21 (84%) | 5 (100%) | 5 (100%) | 5 (100%) | 2 (100%) | 8 (80%) | 4 (100%) | 1 (50%) |
| In TRT | 2 (4%) | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 1 (50%) |
| In Recur TRT | 2 (4%) | 1 (4%) | 0 | 0 | 0 | 0 | 2 (20%) | 0 | 0 |
| In Additional TRT | 1 (2%) | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| Death | 4 (8%) | 3 (12%) | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| Current Age, Median (years), range | 26 (4–68) | 14 (2–54) | 23 (16–35) | 20 (7– 53) | 48 (36–57) | 40 (18–62) | 40 (17–61) | 18 (13–30) | 15 (11–19) |
| Germline DICER1 mutation (n=89) | 25/41 | 0/19 | 3/5 | 0/5 | 0/5 | 0/1 | 0/9 | 0/3 | 0/1 |
| Tumor DICER1 mutation (n=48) | 36/37 | 0/2 | 4/4 | 0/0 | 0/1 | 0/1 | 0/1 | 0/0 | 0/0 |
SLCT= Sertoli-Leydig cell tumor, JGCT= juvenile granulosa cell tumor, GAB= gynandroblastoma, AGCT= adult granulosa cell tumor, NOS= not otherwise specified. NED= no evidence of disease, TRT = treatment, Recur= recurrence