Abstract
Neuroendocrine neoplasms (NENs) are particularly rare in all sites of the gynecological tract and include a variety of neoplasms with variable prognosis, dependent on histologic subtype and site of origin. Following the expert consensus proposal of the International Agency for Research on Cancer (IARC), the approach in the latest World Health Organization (WHO) Classification System of the Female Genital Tumours is to use the same terminology for NENs at all body sites. The main concept of this novel classification framework is to align it to all other body sites and make a clear distinction between well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs). The previous WHO Classification System of the Female Genital Tumours featured more or less the same principle, but used the terms ‘low-grade neuroendocrine tumor’ and ‘high-grade neuroendocrine carcinoma’. Regardless of the terminology used, each of these two main categories include two distinct morphological subtypes: NETs are represented by typical and atypical carcinoid and NEC are represented by small cell neuroendocrine carcinoma (SCNEC) and large cell neuroendocrine carcinoma (LCNEC). High-grade NECs, especially small cell neuroendocrine carcinoma tends to be more frequent in the uterine cervix, followed by the endometrium, while low-grade NETs usually occur in the ovary. NENs of the vulva, vagina and fallopian tube are exceptionally rare, with scattered case reports in the scientific literature.
Highlights
Neuroendocrine neoplasms (NENs) derive from endocrine cells of the diffuse neuroendocrine system, arising mainly in the gastrointestinal tract, lungs and pancreas [1]
High-grade neuroendocrine carcinomas (NECs), especially small cell neuroendocrine carcinoma tends to be more frequent in the uterine cervix, followed by the endometrium, while low-grade neuroendocrine tumors (NETs) usually occur in the ovary
In this article we review the scientific progress achieved in neuroendocrine neoplasms of the gynecologic tract, since their discovery till today and highlight the latest advancements regarding novel diagnostic modalities, modern histopathologic classifications and most effective therapeutic concepts
Summary
Neuroendocrine neoplasms (NENs) derive from endocrine cells of the diffuse neuroendocrine system, arising mainly in the gastrointestinal tract, lungs and pancreas [1]. They are rarely encountered in the gynecological tract, and when they are, they involve the ovary or cervix [2]. According to the latest World Health Organization (WHO) Classification System of the Female Genital Tumours, NETs are low-grade or intermediate-grade epithelial neoplasms with morphological and immunohistochemical features of neuroendocrine differentiation. NECs are high-grade (grade 3) and poorly differentiated epithelial neoplasms which show variable morphological and immunohistochemical features of neuroendocrine differentiation. Similar to other body sites, NECs are classified, based on cell morphology, into small cell neuroendocrine carcinomas (SCNEC) and large cell neuroendocrine carcinomas (LCNEC)
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