Abstract: Ovarian clear cell carcinoma（OCCC） is a special histological type in epithelial ovarian cancers（EOC）. The incidence of OCCC is about 5 to 25 percent of EOC. In early stage，OCCC often has better prognosis，furthermore，some patients do not need secondary treatment. But the advanced stage often has poor prognosis，as to its insensitive to conventional chemotherapy. OCCC is closely related to the occurrence of endometriosis. Recent studies have shown that tumor suppressor genes involved in chromatin remodeling-AT-rich binding domain 1A gene（ARID1A） is related to the pathogenesis of OCCC. ARID1A protein is a subunit of SWI/SNF complex, which plays important roles in replication，transcription, repair, and restruction of DNA, inhibits cell proliferation and tumor growth. Mutations of ARID1A are often seen in gynecologic cancers, especially in OCCC （46%-57%）. Mutations of ARID1A arise apoptosis and mismatch repair defects, through upregulation of the PI3K/Akt pathway, particularly through mutations of PI3KCA and inactivation of PTEN, and also which can enhance the activity of pathways involved in angiogenesis and have impact on HER2. The depth and comprehensive study of ARID1A gene can provide a basis for earlier diagnosis and effective intervention molecular target therapy. This article is about the role of ARID1A gene in OCCC.

Key words: Ovarian neoplasms, Adenocarcinoma, clear cell, Mutation, ARID1A