Neuroendocrine tumors of the gynecological tract: A narrative literature review.

Abstract

Neuroendocrine neoplasms (NENs) of the gynecological tract are a rare, heterogenous and aggressive group of neoplasms, with high recurrence rates and poor prognosis. In this review we focus on NENs of the gynecological system emphasizing the classification, epidemiological and clinical characteristics of NENs across the gynecological tract (cervix, endometrium, ovary, vagina, and vulva), risk/prognostic factors, pathology and molecular biology (including actionable genomic mutations), imaging, staging and the most effective treatment modalities in the "standard of care" approach as well as the pipeline products. We also focused on metastatic spreading patterns of gynecological NENs. We searched for all available literature reviews, interventional studies, short series, case reports and meta-analyses published from 1990 to 2024. Deteriorated survival rate is essentially impacted by early development of lymph node, distant organ metastases and vascular propagation toward rapid extra-pelvic metastasis to the brain, liver, lung, bone marrow, lymph nodes and bones. Management of NENs needs to be customized on a case-based manner and comprises a multidisciplinary approach that involves gynecologists, surgeons, medical oncologists, radiologists, radiation oncologists, nuclear medicine specialists, pathologists, molecular biologists, qualified nurses etc. The treatment of extrapulmonary NENs arising in the female genital tract is basically extrapolated from that for small cell lung cancer. This includes, but is not limited to: surgery, chemotherapy, radiotherapy, peptide receptor radionuclide therapy, somatostatin analogs, and immunotherapy as well as investigational drugs in rare prospective clinical trials. Establishing modern therapeutic thesaurus is conditioned by the existence of well-designed clinical trials targeting a tumor's genomic profile and the incorporation of these data into the actual treatment landscape.