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General
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- Resemble fibroepithelial stromal polyps of vagina, cervix and vulva, but with bizarre morphology, frequent mitoses (>10/10 HPF), atypical mitotic figures or hypercellularity
- Median age 32, range 16-75 years
- Often in pregnant patients
- May recur locally; no metastases
- Positive margin status, which is common, apparently not associated with recurrence
Gross description
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- Often multiple lesions, particularly in pregnant women
Positive stains
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Differential diagnosis
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- Aggressive angiomyxoma: deep, prominent vascular pattern cuffed by myoid bundles
- Angiomyofibroblastoma: well circumscribed subserosal nodule, no atypia, stromal cells cluster around vessels, which usually have delicate walls
- Botyroid embryonal rhabdomyosarcoma: early childhood, submucosal hypercellular zone/cambium layer, rhabdomyoblasts, myoglobin+, myogenin+
- Cellular angiofibroma: well circumscribed, less polypoid, diffusely vascular with hyalinized walls, no atypical stromal cells, desmin-
- DFSP: CD34+, desmin-, storiform throughout
- Leiomyosarcoma: clear boundary of tumor cells with epithelium, smooth muscle differentiation
- Low grade endometrial stromal sarcoma: vessels resemble spiral arterioles, no central vascular core, thick bands of collagen in starburst pattern, dot like staining of desmin or keratin
- MPNST: perivascular accentuation, 50% are S100+
End of Vulva > Benign or non-neoplastic tumors > Pseudosarcomatous fibroepithelial stromal polyps
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