Clinical features |
|
|
Age (years)
|
16 – 86 (Median 45) |
16 – 70 (Median 37) |
Size (cm)
|
< 5 (0.5 – 23) |
>5 (1-60) |
Location
|
Female pelvis and perineum: predominantly vulvar/vaginal |
Female pelvis and perineum |
Onset
|
1 month to 8 years |
1 month – 5 years |
Predominantly 1-2 years |
Predominantly < 1 year |
Recurrence
|
None reported |
33-72% |
Pathology |
|
|
Low-power features
|
Well-defined |
Infiltrative |
No entrapment of mucosal glands or nerve bundles |
Entrapped mucosal glands and nerve bundles |
Alternating hypocellular and hypercellular areas |
Stromal cells distributed throughout |
Vasculature
|
Abundant thin-walled vessels, mostly capillary-like |
Small to medium-sized vessels, mostly thick-walled or hyalinized |
Stromal cells
|
Abundant |
Low cellularity |
Spindle, plump spindle, or oval |
Thin delicate spindle or stellate |
Perivascular aggregation |
Mitotic figures typically absent |
Mitotic figures typically absent |
|
Stroma
|
Mucin-poor, containing delicate, wavy collagen fiber |
Myxoid, hyaluronic-acid rich |
Immunostaining |
|
|
Desmin
|
Positive (50-60%) |
Positive (Up to 73%) |
α
–SMA
|
Negative (positive in up to 15%) |
Positive |
S-100
|
Negative |
Negative |
Vimentin
|
Positive |
Positive |
Estrogen receptor
|
Positive |
Positive |
Progesterone receptor
|
Positive |
Positive |