Vulvar Adenocarcinoma With Neuroendocrine Differentiation: A Case Report

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Vulvar cancer makes up 3% to 5% of all female genital tract cancers. Of those, most are squamous cell carcinomas. Primary vulvar adenocarcinomas make up 1% of all vulvar cancers and may arise from skin adnexal structures, anogenital mammary-like glands, vestibular glands, or Bartholin glands.1 Vulvar adenocarcinomas are categorized into the following three subtypes: sweat gland carcinomas, primary “mammary-like” adenocarcinomas, and extramammary Paget disease (EMPD).2 However, the true histologic origins of these tumors are poorly understood. Extramammary Paget disease is thought to be derived from eccrine, apocrine, and mammary-like glands or related pluripotent germinative cells. Van der Putte and van Gorp3 previously suggested that the three categories should be unified.
Previous case reports have described neuroendocrine differentiation in vulvar mucinous adenocarcinoma,4–6 but there are no known cases of nonmucinous vulvar adenocarcinoma with neuroendocrine differentiation. We present a case and a review of the literature. Only consent for treatment was obtained, and therefore, the description has been deidentified to protect privacy.

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