AbstractBackground: Skin adnexal neoplasms of the vulva are uncommon, and malignant adnexal neoplasms of the vulva are rare. Case: A case of syringomatous eccrine carcinoma arising in the perineum is presented, and the literature reviewed. Conclusions: Non-squamous malignancy of the vulva should be considered. All suspicious lesions of the vulva should be biopsied.
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