Table 1.
Clinicopathologic features of GREB1-rearranged uterine sarcoma
| Case number | Age (years) | Tumor size (cm) | Microscopic tumor margins | Tumor architecture | Cytomorphology | Mitosis (per 10 HPF) | LVIc | Necrosis | Fusion gene | Pathology diagnosis | Stage | Status of the disease |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Cheng-Han et al. (2019) | ||||||||||||
| 1 | 56 | 10.0 | Well demarcated | Diffuse, fascicular, trabecular/corded | Epithelioid and spindle, occasional rhabdoid features. | 8 | – | – | GREB1-NCOA2 | UUS-Ub | IB | NEDd at 0.75 mo |
| 2 | 60 | 14.9 | Generally well-defined; rare foci of tongue-like protrusions | Diffuse, trabecular/corded, fascicular | Epithelioid and spindle | 14 | + | + | GREB1-NR4A3 | GREB1-regrranged sarcoma | IB | NED at 5 mo |
| 3 | 68 | 8.5 | Myometrial delicate cell cords invasion | Trabecular/corded, diffuse, nested | Epithelioid | 7 | – | – | GREB1-SS18 | Atypical mesenchymal tumor | IB | NED at 6 mo |
| 4 | 65 | 4.2 | Rare foci tongue-like protrusion | Trabecular/corded, diffuse, nested, fascicular; multifocal tubular/retiform structures | Epithelioid and spindle, rare lipid-laden cells and Leydig-like cells | 2 | – | – | GREB1-NCOA1 | GREB1-regrranged sarcoma | IA | Recent case |
| Croce et al. (2019) | ||||||||||||
| 5 | 70 | 10.0 | Relatively well demarcated | Diffuse, nested, trabecular | Epithelioid, focal rhabdoid appearance | 1 | – | NA | GREB1-CTNNB1 | UTROSCT | IB | Pelvic dissemination (17 mo); lung metastasis (30 mo) |
| Brunetti et al. (2018) | ||||||||||||
| 6 | 51 | 6.5 | Infiltrative | Primary tumor: diffuse fascicular; recurrent tumor: solid growth pattern | Primary tumor: spindle and polygonal cells displaying pronounced atypia; Recurrent tumor: predominantly epithelioid | multiple mitotic figuresa | + | + | GREB1-NCOA2 | High-grade endometrial sarcoma/sarcoma, not otherwise classifiable | IB | lung metastasis (24 and 132 y) |
| Dickson et al. (2019) | ||||||||||||
| 7 | 68 | 0.7–3.3a | NA | Fascicles, focal tubular pattern | Spindle and epithelioid | 0–1 | NA | – | GREB1-NCOA2 | UTROSCT | I | NA |
| Goebel et al. (2019) | ||||||||||||
| 8 | 71 | 6.2 | Infiltrative | Nested, corded | Spindle and epithelioid | 1 | – | NA | GREB1-NCOA1 | UTROSCT | NA | NED at 54.3 mo |
| 9 | 71 | NA | Infiltrative | Sertoliform, retiform | Spindle and epithelioid | < 1 | – | NA | GREB1-NCOA1 | UTROSCT | NA | NA |
| 10 | 71 | 13.0 | Infiltrative | Corded, trabecular, sertoliform, retiform | Spindle and epithelioid | 1 | – | NA | GREB1-NCOA2 | UTROSCT | NA | Recurrent to pelvis (66 mo) |
| 11 | 74 | at least 2.5 | Infiltrative | Sertoliform | Spindle and epithelioid | 3 | – | NA | GREB1-NCOA1 | UTROSCT | NA | NA |
| 12 | 61 | 8.0 | Infiltrative | Nested, corded, whorled | Spindle and epithelioid | 2 | – | NA | GREB1-NCOA1 | UTROSCT | NA | NED at 10 mo |
| Present case | ||||||||||||
| 13 | 57 | 10.0 | Infiltrative | Diffuse, nested, trabecular/corded, and focal anastomotic glandular | Spindle and epithelioid | 3 | – | – | GREB1-NCOA2 | UTROSCT | IB | Recurrent to pelvis (30 mo) |
aExact data not provided
bUUS-U undifferentiated uterine sarcoma with nuclear uniformity
cLVI lymphovascular invasion
dNED no evidence of disease