Neuroendocrine tumors of the female genital tract

Both primary and secondary gynecologic neuroendocrine tumors (NET) are uncommon, and they make up around 2 % of gynecologic cancers. The literature is scarce and mostly limited to case reports and miniseries in the female genital tract. NET originates from diffuse neuroendocrine cell system and may arise in any anatomical site of the female genital tract, including the vulva, vagina, cervix, endometrium, and ovary. They comprise a spectrum of tumors with variable biologic potential. Due to the rarity of such tumors, general pathologists’ experience is limited and these may present diagnostic challenges. Currently the nomenclature employed is still that of the pulmonary classification systems, carcinoid, atypical carcinoid, and small-and large-cell neuroendocrine carcinoma. The terminology correlates with grades 1-3 of the WHO gastrointestinal neuroendocrine tumor classification. The proliferative rate is assessed based on mitotic index, and usage of Ki-67 staining is not a routine practice. In this review we cover selected neuroendocrine tumors of the gynecologic tract and available molecular findings related to the topic.