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Their clinical care lacks standardization, often with incidental discovery after surgery for presumed epithelial tumors. This review synthesizes evidence from 2014–2024 to summarize epidemiology, pathogenesis, presentation, diagnosis, treatment, and prognosis, emphasizing unmet needs and the need for multidisciplinary, multicenter research.",{"@graph":69,"@context":123},[70,84,106],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/ovarian-neuroendocrine-neoplasms-challenges-and-future-perspectives/376472/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/ovarian-neuroendocrine-neoplasms-challenges-and-future-perspectives/376472.png","ImageObject",300,407,{"name":92,"@type":93},"dhado","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-27","2026-09-24",true,{"@type":102,"interactionType":103,"userInteractionCount":105},"InteractionCounter",{"@type":104},"ViewAction",14,{"@type":107,"mainEntity":108},"FAQPage",[109,115,119],{"name":110,"@type":111,"acceptedAnswer":112},"What are ovarian neuroendocrine neoplasms and how rare are they?","Question",{"text":113,"@type":114},"They are extremely rare ovarian tumors, representing less than 1% of all ovarian neoplasms and under 5% of all neuroendocrine tumors. The review describes two main histological subtypes: well-differentiated carcinoids and poorly differentiated neuroendocrine carcinomas.","Answer",{"name":116,"@type":111,"acceptedAnswer":117},"How are ovarian neuroendocrine neoplasms diagnosed?",{"text":118,"@type":114},"Diagnosis typically follows the same work-up protocol as epithelial ovarian cancer, with the neuroendocrine nature often recognized only after postoperative histologic examination. Clinical presentations can range from asymptomatic masses to hormone-related syndromes that mimic other ovarian conditions.",{"name":120,"@type":111,"acceptedAnswer":121},"Why are treatment strategies challenging and what does the review recommend?",{"text":122,"@type":114},"Treatment is largely empirical and often extrapolated from extra-ovarian neuroendocrine tumor data because specific guidelines are lacking. The review highlights the need for greater clinical awareness, multidisciplinary management, and multicenter research to build evidence-based protocols.","https://schema.org",{"og:url":83,"og:type":125,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":127,"canonical":83},"index,follow",{"doc_id":129,"site_id":62},376472,1790506667,{"code":4,"msg":5,"data":132},{"doc_id":129,"user_id":133,"nickname":92,"user_avatar":134,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":135,"file_id":136,"file_url":137,"file_type":138,"file_size":139,"view_count":105,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":56,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":145},5909893218464,"https://ap-avatar.wpscdn.com/davatar_085a072bc5b1113ac321206ff7593b45","Review  \nOvarian Neuroendocrine Neoplasms: Challenges and Future Perspectives  \nValentina Di Vito 1, Gabriele Veroi 1,2, Laura Rizza 1, Francesca Rota 1, Andrea Baiocchini 3,  \nMaria Cristina Macciomei 3, Carla Lubrano 2, Anna La Salvia 4, Andrea Lania 5, Lucia Rosalba Grillo 3, Silvia Migliaccio 2, Guido Rindi 6 and Roberto Baldelli 1, *  \nAcademic Editor: Pedro Iglesias  \nReceived: 30 October 2025  \nRevised: 28 November 2025  \nAccepted: 6 December 2025  \nPublished: 13 December 2025  \nCitation: Di Vito, V.; Veroi, G.; Rizza, L.; Rota, F.; Baiocchini, A.; Macciomei, M.C.; Lubrano, C.; La Salvia, A.; Lania, A.; Grillo, L.R.; et al. Ovarian Neuroendocrine Neoplasms:  \nChallenges and Future Perspectives. J. Clin. Med. 2025, 14, 8833. [https://](https://)[ ](https://)[doi.org/10.3390/jcm14248833](doi.org/10.3390/jcm14248833)  \n[Copyright:](Copyright:) © 2025 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ([https://creativecommons.org/](https://creativecommons.org/)[ ](https://creativecommons.org/)[licenses/by/4.0/](licenses/by/4.0/)) .  \n1 Department of Endocrinology, San Camillo-Forlanini Hospital, 00152 Rome, Italy; [valentinadivito2@gmail.com](valentinadivito2@gmail.com) (V.D.V.); [gabriele.veroi@uniroma1.it](gabriele.veroi@uniroma1.it) (G.V.); [laura_rizza@hotmail.it](laura_rizza@hotmail.it) (L.R.); [francescarota73@tiscali.it](francescarota73@tiscali.it) (F.R.)  \n2 Department of Experimental Medicine, Section of Medical Pathophysiology, Food Science and Endocrinology, Sapienza University of Rome, 00185 Rome, Italy; [carla.lubrano@uniroma1.it](carla.lubrano@uniroma1.it) (C.L.); [silvia.migliaccio@uniroma1.it](silvia.migliaccio@uniroma1.it) (S.M.)  \n3 Department of Anatomic Pathology, San Camillo-Forlanini Hospital, 00152 Rome, Italy; [abaiocchini@scamilloforlanini.rm.it](abaiocchini@scamilloforlanini.rm.it) (A.B.); [mmacciomei@scamilloforlanini.rm.it](mmacciomei@scamilloforlanini.rm.it) (M.C.M.); [lrgrillo@scamilloforlanini.rm.it](lrgrillo@scamilloforlanini.rm.it) (L.R.G.)  \n4 National Center for Drug Research and Evaluation, National Institute of Health (ISS), 00161 Rome, Italy; [anna.lasalvia@iss.it](anna.lasalvia@iss.it)  \n5 Department of Endocrinology, Diabetology and Andrology, IRCCS Humanitas Research Hospital,  \n20089 Rozzano, Italy; [andrea.lania@hunimed.eu](andrea.lania@hunimed.eu)  \n6 Department of Anatomic Pathology, Università Cattolica del Sacro Cuore, 00168 Rome, Italy; [guido.rindi@policlinicogemelli.it](guido.rindi@policlinicogemelli.it)  \n* Correspondence: [rbaldelli@scamilloforlanini.rm.it](rbaldelli@scamilloforlanini.rm.it)  \nAbstract  \nBackground: Ovarian neuroendocrine neoplasms (O-NENs) are extremely rare, representing less than 1% of all ovarian neoplasms and under 5% of all neuroendocrine tumors (NETs) . They encompass two primary histological subtypes: well-differentiated carcinoids and poorly differentiated neuroendocrine carcinomas, which display distinct biological behaviors and prognoses. The ovary can also be a site of metastasis from extraovarian NETs. Owing to their rarity, clinical management lacks standardization, and diagnosis is often incidental following surgery for presumed epithelial ovarian neoplasms. Objectives: This review aims to provide an updated synthesis of current evidence on the epidemiology, pathogenesis, clinical presentation, diagnosis, treatment strategies, and prognosis of O-NENs, highlighting unmet clinical needs. Methods: A literature search was performed on PubMed for the years 2014–2024 using the keywords: “ovarian neuroendocrine tumor”,“ovarian neuroendocrine neoplasm”,“ovarian neuroendocrine carcinoma”, and “ovarian carcinoid”. Only articles published in English were considered. Given the rarity of the disease, in addition to meta-analyses and systematic reviews, relevant case reports and case series were also included to provide a ","cbCaisfwub4H8Q95","https://ap.wps.com/l/cbCaisfwub4H8Q95","pdf",1071550,"English","# Abstract\n# Background\n## Objectives\n## Methods\n## Results\n## Conclusions\n# Introduction","[{\"question\":\"What are ovarian neuroendocrine neoplasms and how rare are they?\",\"answer\":\"They are extremely rare ovarian tumors, representing less than 1% of all ovarian neoplasms and under 5% of all neuroendocrine tumors. The review describes two main histological subtypes: well-differentiated carcinoids and poorly differentiated neuroendocrine carcinomas.\"},{\"question\":\"How are ovarian neuroendocrine neoplasms diagnosed?\",\"answer\":\"Diagnosis typically follows the same work-up protocol as epithelial ovarian cancer, with the neuroendocrine nature often recognized only after postoperative histologic examination. Clinical presentations can range from asymptomatic masses to hormone-related syndromes that mimic other ovarian conditions.\"},{\"question\":\"Why are treatment strategies challenging and what does the review recommend?\",\"answer\":\"Treatment is largely empirical and often extrapolated from extra-ovarian neuroendocrine tumor data because specific guidelines are lacking. The review highlights the need for greater clinical awareness, multidisciplinary management, and multicenter research to build evidence-based protocols.\"}]","Ovarian Neuroendocrine Neoplasms - Challenges and Future Perspectives | PDF",1790219345,48]