Background/Objectives: Vaginal adenocarcinoma is a rare malignancy, accounting for less than 10% of all primary vaginal cancers. It predominantly affects older women but can also occur in younger populations, particularly in association with in utero diethylstilbestrol (DES) exposure. Given its rarity, evidence regarding the optimal management of vaginal adenocarcinoma remains limited. This review aimed to summarize the current understanding of vaginal adenocarcinoma, covering the epidemiology, etiology, diagnostic approaches, treatment modalities, prognosis, and areas requiring further investigation thereof. Methods: We conducted a search for the term “vaginal adenocarcinoma” in the PubMed, Scopus, and Web of Science databases from January 2016 to 28 April 2025. Results: Overall, 83 articles were included in the final review. Among them, 21 cases of vaginal adenocarcinoma were reported. Vaginal adenocarcinoma demonstrates a bimodal age distribution, with clear cell histology commonly linked to DES exposure and endometrioid or mucinous types seen in older patients. Risk factors include DES exposure, chronic inflammation, and human papillomavirus (HPV) infection. The diagnosis relies on a pelvic examination, imaging, and biopsy. Treatment typically involves surgery, radiotherapy, or a combination thereof, tailored to the stage and location, with chemotherapy reserved for advanced cases. The prognosis depends on the histologic subtype, tumor size, stage, and treatment response, with early-stage disease generally associated with better outcomes. Conclusions: Improved awareness of risk factors and early diagnostic strategies is critical to optimize patient outcomes. Research is needed to refine treatment protocols, explore targeted therapies and immunotherapy, and investigate the molecular underpinnings of vaginal adenocarcinoma, particularly non-DES-associated types.
**背景/目的:** 阴道腺癌是一种罕见的恶性肿瘤,在所有原发性阴道癌中占比不足10%。其好发于老年女性,但也可发生于年轻人群,尤其与宫内己烯雌酚暴露相关。鉴于其罕见性,关于阴道腺癌最佳治疗方案的证据仍然有限。本综述旨在总结当前对阴道腺癌的认识,涵盖其流行病学、病因学、诊断方法、治疗模式、预后以及需要进一步研究的领域。 **方法:** 我们在PubMed、Scopus和Web of Science数据库中检索了2016年1月至2025年4月28日期间包含"阴道腺癌"一词的文献。 **结果:** 共有83篇文章被纳入最终综述。其中报告了21例阴道腺癌病例。阴道腺癌呈现双峰年龄分布,透明细胞型通常与DES暴露相关,而子宫内膜样型或黏液型多见于老年患者。危险因素包括DES暴露、慢性炎症和人乳头瘤病毒感染。诊断依赖于盆腔检查、影像学检查和活检。治疗通常包括手术、放疗或两者联合,根据分期和部位进行个体化选择,化疗则用于晚期病例。预后取决于组织学亚型、肿瘤大小、分期和治疗反应,早期疾病通常预后较好。 **结论:** 提高对危险因素的认识和早期诊断策略对于优化患者预后至关重要。需要开展研究以完善治疗方案、探索靶向治疗和免疫疗法,并深入研究阴道腺癌,特别是非DES相关类型的分子基础。
Vaginal Adenocarcinoma: A Review of a Rare Gynecologic Cancer