Summary of clinicopathological features of PEComas of the gynaecological tract
| Uterine corpus | Cervix | Vagina | Adnexa | Broad ligament | Vulva | |
|---|---|---|---|---|---|---|
| N | 78 | 11 | 7 | 6 | 5 | 1 |
| Age at diagnosis, median (range), years | 47.5 (9–79) | 46 (25–61) | 28 (6–57) | 49 (33–63) | 25 (24–57) | 20 |
| Associations | ||||||
| TSC | 5 (6%) | 1 (9%) | – | 2 (33%) | – | |
| Other PEComa family tumours | LAM (n=3) | – | – | – | – | |
| PEComatosis | 4 (5%) | 1 (9%) | – | – | – | |
| Tumour size, median (range), cm | 5 (0.2–30) | 3.9 (1–12) | 3 (1.5–9) | 4.2 (2.5–15) | 11.5 (4–17) | 2 |
| (n=67) | (n=9) | (n=5) | (n=6) | (n=5) | ||
| Morphology | ||||||
| Sclerosing PEComa | 9 (12%) | 0 | 0 | 2 (33%) | 1 (20%) | |
| Cell shape | – | |||||
| Epithelioid | 43/74 (58%) | 6/7 (86%) | 2/4 (50%) | |||
| Spindle | 1/74 (1%) | 6/10 (60%) | – | 3/6 (50%) | 1/4 (25%) | |
| Epithelioid+spindle | 30/74 (41%) | 4/10 (40%) | 1/7 (14%) | 3/6 (50%) | 1/4 (25%) | |
| Necrosis | 31/74 (42%) | 4/10 (40%) | 1/5 (20%) | 2/6 (33%) | 3/4 (75%) | – |
| Nuclear atypia | Significant, severe or extensive in 25/62 (40%) | Moderate to severe in 7/10 (70%) | Severe atypia not seen (5/5, 0%) | ‘Severe’/‘significant’ atypia in 4/6 (67%) | Varying from none to severe | None |
| Mitotic activity | ≤1 (38, 52%) Rare (7, 10%) 2–222 per 50 HPF (28, 38%) (n=73) | ‘Zero’, ‘rare’ or ≤1/50 HPF (8/9, 89%) | Absent or ‘rare’ 4/5 (80%)* | Variable: ≤1 (3/6) to 97/50 HPF | Rare | |
| IHC | (see also table 2) | |||||
| HMB-45 | 71/72 (99%) | 8/8 (100%)† | 6/6 (100%)‡ | 6/6 (100%) | 4/4 (100%) | 1/1 (100%) |
| Melan-A | 21/46 (46%) | 4/5 (80%) | 1/4 (25%) | 3/3 (100%)§ | 1/3 (33%) | |
| MiTF | 14/21 (67%) | 0/1 (0%) | 1/1 (100%)¶ | |||
| SMA | 53/68 (80%) | 5/8 (63%) | 2/4 (50%) | 4/5 (80%) | 4/4 (100%)** | |
| Desmin | 39/62 (63%) | 3/5 (60%) | 0/2 (0%) | 4/6 (67%) | 1/2 (50%)†† | 0/1 (0%) |
| Caldesmon | 17/22 (77%) | 2/2 (100%) | ||||
| Cytokeratin | 2/43 (5%) | |||||
| Oestrogen receptor (ER) | 10/19 (53%) | |||||
| Progesterone receptor (PR) | 11/13 (85%) | |||||
| PAX8 | 0 | |||||
| CD10 | 4/28 (14%) | |||||
| CD34 | 0 | |||||
| Vimentin | 11/18 (61%) | |||||
| Inhibin | 1/20 (5%) | |||||
| Follow-up | ||||||
| Duration, median (range), months | 20 (1.5–168) | 28 (9–42) | 14.5 (3–54) | 9 (4–72) | 13.5 (11–18) | 48 |
| Died of disease | 10/63 (16%) | 1/9 (11%)78 | – | 1/4 (25%) | – | – |
| No evidence of disease | 44/63 (70%) | 8/9 (89%) | 6/7 (86%) | 3/4 (75%) | 2/4 (50%) | 1/1 (100%) |
| Alive with disease | 9/63 (14%) | – | 1/7 (14%) | 2/4 (50%) | – | |
*A single transcription factor E3 translocation-associated case had a mitotic count of five per 50 HPF.
†Strong or diffuse in six of six cases.
‡Strong or diffuse in three of three cases.
§Focally weakly positive in two of three cases and was strongly positive in one of three cases.
¶Weakly positive in less than half of tumour cells.
**Focally positive in two tumours.
††Focally positive.
HPF, high power field; IHC, immunohistochemical; LAM, lymphangioleiomyomatosis; MiTF, microphthalmia-associated transcription factor; PEComas, perivascular epithelioid tumours; TSC, tuberous sclerosis complex.