源自妇科（包括卵巢、子宫内膜、子宫颈、外阴或阴道）的透明细胞癌代表罕见但具有临床意义的实体，具有有趣的重叠特征。从流行病学角度来看，透明细胞癌好发于亚洲种族女性，并且通常与先前或同时诊断的子宫内膜异位症有关。病理上，尽管起源于不同的原发器官，但妇科透明细胞癌在传统组织病理学上表现出相似的形态学和免疫表型特征，如免疫组化上表达Napsin A和HNF1b，但不表达WT1。这些癌症的详细描述的分子特征包括 ARID1A、PIK3CA 和/或 PTEN 等基因的反复突变，尽管不同解剖部位存在显着差异。由于透明细胞癌相对罕见且亚型特异性临床试验有限，因此最佳治疗管理仍然具有挑战性。手术仍然是治疗的基石，通常辅以全身化疗。然而，近年来出现了针对血管生成或免疫微环境的有前景的药物，取得了临床成功，并可能重塑妇科透明细胞癌的治疗格局。本综述总结了不同解剖来源的透明细胞癌在流行病学、病理学、分子特征和治疗方法方面的共性和差异，为这些恶性肿瘤的进一步研究和专门的治疗干预奠定了基础。

Clear cell carcinomas arising from the gynecological tract (including from the ovary, endometrium, cervix, vulva or vagina) represent rare but clinically significant entities with intriguing overlapping characteristics. Epidemiologically, clear cell carcinomas exhibit a predilection for women of Asian ethnicity and are often associated with a previous or synchronous diagnosis of endometriosis. Pathologically, despite originating from different primary organs, clear cell carcinomas of the gynecological tract show a similar morphological and immunophenotypic features on traditional histopathology, such as the expression of Napsin A and HNF1b on immunohistochemistry, without the expression of WT1. Well-described molecular characteristics of these cancers include recurrent mutations in genes such as ARID1A, PIK3CA, and/or PTEN, although significant variations exist across the different anatomical sites. Therapeutically, optimal management remains challenging due to the relative rarity of clear cell carcinomas and limited subtype-specific clinical trials. Surgery remains the cornerstone of treatment, often complemented by systemic chemotherapy. However, promising drugs targeting angiogenesis or the immune microenvironment have emerged in recent years, leading to clinical successes and are likely to reshape the therapeutic landscape of gynecological clear cell carcinoma. This review summarizes the commonalities and disparities in terms of epidemiology, pathology, molecular features and therapeutic approach, amongst clear cell carcinomas of different anatomical origin, offering a foundation for further research and dedicated therapeutic interventions for these malignancies.