Neuroendocrine tumors of the gynecologic tract: select topics

Tumors of the diffuse neuroendocrine cell system (DNES) may arise in any component of the gynecologic tract, including the vulva, vagina, cervix, endometrium, and ovary. Overall such tumors in the gynecologic tract are rare, constituting only 2% of gynecologic cancers, comprising a spectrum of tumors of variable biologic potential. Due to the rarity of such tumors, pathologists experience may be limited and these may present diagnostic challenges. Currently the nomenclature employed is still that of the pulmonary classification systems, carcinoid, atypical carcinoid, small and large cell neuroendocrine carcinoma that broadly correlates to low/grade 1, intermediate/grade 2, and high grade/grade 3 of the WHO gastroenteropancreatic neuroendocrine tumors classification. Furthermore in keeping with the lung, proliferative rate is assessed based on mitotic index rather than Ki-67 staining. In this review we cover select neuroendocrine tumors of the gynecologic tract.