女性阴道癌 Paget 病（VPD）是一种罕见的阴道皮肤腺癌，约占所有阴道肿瘤的1-2％，主要影响绝经后的女性。临床表现通常不具体，类似慢性红斑性皮肤病变; 因此，诊断常常困难和延迟。虽然 VPB 通常在局部晚期被诊断，并且具有高复发率，但预后总体有利，5年生存率近90％。由于证据有限且质量较差，因此对于最佳管理没有全球共识。因此，我们通过主要电子数据库进行了系统综述，以加深对这种罕见疾病的现有知识，并讨论可用的治疗策略。广泛的手术切除是标准的治疗方法，并且应根据肿瘤位置/扩展和患者的表现状况进行调整。目标是完全去除肿瘤并取得清晰的边缘，从而降低局部复发率。非手术治疗，如放射治疗，化学治疗和局部治疗，可以考虑，尤其是在无法切除和复发疾病的情况下。在没有明确建议的情况下，决策过程应个体化，还考虑新出现的分子靶点，例如HER2和PD-L1，这可能为未来的靶向治疗铺平道路。当前的综述旨在提高对这种罕见疾病的认识，并鼓励国际协作收集更大规模，高质量的证据，并标准化治疗。

Vulvar Paget's disease (VPD) is a rare form of cutaneous adenocarcinoma of the vulva, which accounts for about 1-2% of all vulvar neoplasms and mainly affects post-menopausal women. The clinical presentation is usually non-specific and mimics chronic erythematous skin lesions; therefore, the diagnosis is often difficult and delayed. Although VPD is typically diagnosed at a locally advanced stage and has a high recurrence rate, the prognosis is overall favorable with a 5-year survival of nearly 90%. Due to the limited and poor-quality evidence, there is no global consensus on optimal management. Therefore, we performed a systematic review of the literature through the main electronic databases to deepen the current knowledge of this rare disease and discuss the available treatment strategies. Wide surgical excision is recommended as the standard-of-care treatment and should be tailored to the tumor position/extension and the patient's performance status. The goal is to completely remove the tumor and achieve clear margins, thus reducing the rate of local recurrences. Non-surgical treatments, such as radiotherapy, chemotherapy, and topical approaches, can be considered, especially in the case of unresectable and recurrent disease. In the absence of clear recommendations, the decision-making process should be individualized, also considering the new emerging molecular targets, such as HER2 and PD-L1, which might pave the way for future targeted therapies. The current review aims to raise awareness of this rare disease and encourage international collaboration to collect larger-scale, high-quality evidence and standardize treatment.