Mesenchymal lesions of the vulva include site-specific entities limited to the lower genital tract, as well as a range of non-site-specific tumors that are more common at extragenital sites. Site-specific lesions include fibroepithelial stromal polyp, cellular angiofibroma, angiomyofibroblastoma, and aggressive angiomyxoma. Non-site-specific tumors that may occur in the vulva include those of smooth muscle, skeletal muscle, vascular, neural, adipocytic, and uncertain differentiation. This review discusses both site-specific and non-site-specific vulvar mesenchymal lesions including non-neoplastic proliferations, benign neoplasms, locally aggressive neoplasms with a predilection for local recurrence, neoplasms of indeterminate biologic potential, and frankly malignant neoplasms with a high risk of distant metastasis and death. Accurate diagnosis is essential for proper management, and is facilitated by correlation with clinical findings and targeted application of immunohistochemical and molecular studies.
Keywords: Aggressive angiomyxoma; Angiomyofibroblastoma; Cellular angiofibroma; Fibroepithelial stromal polyp; Soft tissue tumors; Vulva.
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