Lu Chengbin, Fan Die, Xiong Yu, Jiang Xiaoxia, Li Jiangsha, Yang Ping, Shi Yuanlong, Li Hongmei, Li Zheng
Department of Gynecologic Oncology, The Third Affiliated Hospital of Kunming Medical University, Yunnan Cancer Hospital, Peking University Cancer Hospital Yunnan, Yunnan Kunming, 650118, China.
The library of Kunming Medical University, Yunnan Kunming, 650500, China.
BMC Womens Health. 2025 Sep 26;25(1):444. doi: 10.1186/s12905-025-03980-0.
Primary Vaginal Melanoma (PVM) is a rare and highly aggressive malignancy, accounting for only 0.3-0.8% of all female melanomas, with an extremely poor prognosis.
This study aims to present the diagnostic process, surgical management, and postoperative chemotherapy options in a patient initially diagnosed with vaginal neuroendocrine carcinoma, later confirmed to have PVM. Additionally, we provide a systematic review to enhance awareness of PVM, reduce misdiagnosis, and improve early detection.
A systematic review of the literature was conducted, and a case report was analyzed.
A total of 89 publications were reviewed, summarizing the various treatment strategies and survival outcomes for PVM. In the reported case, postoperative pathology confirmed the diagnosis of PVM, differentiating it from primary vaginal neuroendocrine carcinoma. The treatment involved partial vaginal excision and removal of lesions from the posterior vaginal wall, followed by adjuvant chemotherapy with temozolomide and cisplatin.
PVM is highly malignant with a poor prognosis, and surgical resection remains the primary treatment modality; however, its efficacy is limited. While chemotherapy may benefit some patients, the five-year survival rate ranging from 5 to 25%. Therefore, the development of novel therapeutic strategies is crucial. Recent advancements in immunotherapy and targeted therapies targeting BRAF/KIT mutations show promise, but standardized diagnostic and treatment protocols are still lacking. Future multicenter, long-term studies are needed to develop individualized treatment plans based on molecular profiling, providing an evidence-based approach to optimize diagnostic strategies and improve survival rates.
原发性阴道黑色素瘤（PVM）是一种罕见且侵袭性很强的恶性肿瘤，仅占所有女性黑色素瘤的0.3 - 0.8%，预后极差。
本研究旨在介绍一名最初诊断为阴道神经内分泌癌、后来确诊为PVM患者的诊断过程、手术管理及术后化疗方案。此外，我们进行了一项系统综述，以提高对PVM的认识、减少误诊并改善早期检测。
对文献进行了系统综述，并分析了一例病例报告。
共检索了89篇文献，总结了PVM的各种治疗策略和生存结果。在所报告的病例中，术后病理确诊为PVM，将其与原发性阴道神经内分泌癌区分开来。治疗包括部分阴道切除和阴道后壁病变切除，随后采用替莫唑胺和顺铂进行辅助化疗。
PVM恶性程度高，预后差，手术切除仍是主要治疗方式；然而，其疗效有限。虽然化疗可能使部分患者获益，但五年生存率为5%至25%。因此，开发新的治疗策略至关重要。针对BRAF/KIT突变的免疫疗法和靶向疗法的最新进展显示出前景，但仍缺乏标准化的诊断和治疗方案。未来需要开展多中心、长期研究，以根据分子谱制定个体化治疗方案，提供基于证据的方法来优化诊断策略并提高生存率。
