Abstract
Myoepithelial tumors of the vulva are extremely rare, with only 8 cases reported in the literature to date. We report the first case of a high-grade myoepithelial vulvar carcinoma diagnosed in a 35-year-old woman during the 27th week of her pregnancy. The patient initially underwent a wide local excision of the lesion but noted rapid regrowth of the vulvar mass during the next 2 months before her delivery. Shortly thereafter, she underwent a classic radical Taussig-Basset total radical vulvectomy, bilateral superficial and deep inguinal groin node dissection, partial vaginectomy, and reconstruction of the vulva. However, the patient rapidly developed both locoregional and distant mestatatic disease, despite aggressive chemoradiotherapy, and she eventually succumbed to disseminated disease almost 20 months after her initial diagnosis.
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