Mantilidewi Kemala Isnainiasih, Winarno Gatot Nyarumenteng Adhipurnawan, Harsono Ali Budi, Suardi Dodi, Hidayat Yudi Mulyana, Kurniadi Andi, Salima Siti, Erfiandi Febia, Haniah Aini Sofa, Kampan Nirmala Chandralega
Department of Obstetrics and Gynecology, Faculty of Medicine, Universitas Padjadjaran, Dr. Hasan Sadikin General Hospital, Prof Eykman Street No 38, Bandung, 40161, Indonesia.
Gynae-Oncology Unit, Department of Obstetrics and Gynaecology, Hospital Canselor Tuanku Muhriz, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia.
J Ovarian Res. 2025 Jun 9;18(1):125. doi: 10.1186/s13048-025-01701-7.
Neuroendocrine neoplasms (NENs) of the female genital tract are rare, comprising only 1-2% of gynecologic tumors, with ovarian neuroendocrine carcinoma (O-NEC) accounting for less than 1% of all ovarian cancers. Despite its rarity, O-NEC is a highly aggressive tumor with poor prognosis and significant diagnostic complexity, warranting focused clinical attention and demand greater awareness to improve diagnostic and therapeutic strategies.
This systematic review analyzed management paradigm for O-NEC through a comprehensive search on the databases PubMed, Science Direct, Wiley, Springer Link, Google Scholar and Cochrane Central Register of Controlled Trials that was performed on August 1st, 2024.
A comprehensive search on August 1st, 2024, identified 21 eligible studies (6 retrospective cohorts, 12 case reports, 3 case series), encompassing 923 cases of O-NEC. The most common subtypes were small-cell (40%) and large-cell (39.8%) NEC. Most patients presented with advanced-stage (Stage III-IV: 52%). Immunohistochemical markers included synaptophysin (84%), chromogranin A (64.2%), CD56 (69%), and NSE (77.7%). Treatment varied from surgery alone (35%) or surgery plus chemotherapy (32%) being most common. No standardized regimen of chemotherapy was identified with etoposide/cisplatin and paclitaxel/carboplatin were most frequently used. Survival outcomes were poor, with median overall survival ranging from 11 to 23.5 months. The stage at diagnosis was a crucial prognostic factor.
The O-NEC is a rare, heterogeneous malignancy with diverse histopathology, variable immunohistochemical profiles, and generally poor prognosis. Early-stage disease may be managed with surgery alone, while advanced stages require multimodal treatment including surgery with adjuvant platinum-based chemotherapy. Due to limited cases and predominantly retrospective data, standardized diagnostic and treatment protocols are lacking. Prospective multicenter studies and centralized registries are needed to improve understanding and patient outcomes.
女性生殖道神经内分泌肿瘤（NENs）较为罕见，仅占妇科肿瘤的1%-2%，其中卵巢神经内分泌癌（O-NEC）占所有卵巢癌的比例不到1%。尽管O-NEC罕见，但它是一种侵袭性很强的肿瘤，预后较差，诊断复杂性高，需要临床重点关注，并提高认识以改进诊断和治疗策略。
本系统评价通过对2024年8月1日在PubMed、Science Direct、Wiley、Springer Link、谷歌学术和Cochrane对照试验中心注册库等数据库进行全面检索，分析了O-NEC的管理模式。
2024年8月1日的全面检索共纳入21项符合条件的研究（6项回顾性队列研究、12例病例报告、3个病例系列），涵盖923例O-NEC病例。最常见的亚型是小细胞（40%）和大细胞（39.8%）神经内分泌癌。大多数患者为晚期（III-IV期：52%）。免疫组化标志物包括突触素（84%）、嗜铬粒蛋白A（64.2%）、CD56（69%）和神经元特异性烯醇化酶（77.7%）。治疗方式以单纯手术（35%）或手术加化疗（32%）最为常见。未发现标准化的化疗方案，依托泊苷/顺铂和紫杉醇/卡铂使用最为频繁。生存结局较差，总生存中位数为11至23.5个月。诊断时的分期是一个关键的预后因素。
O-NEC是一种罕见的异质性恶性肿瘤，具有多样的组织病理学、可变的免疫组化特征，且总体预后较差。早期疾病可单纯手术治疗，而晚期则需要多模式治疗，包括手术联合铂类辅助化疗。由于病例有限且主要为回顾性数据，缺乏标准化的诊断和治疗方案。需要开展前瞻性多中心研究和建立集中登记系统，以增进了解并改善患者结局。
