Zhu Chenchen, Xu Zhihao, Zhang Tianjiao, Qian Lili, Xiao Weihua, Wei Haiming, Jin Tengchuan, Zhou Ying
Department of Obstetrics and Gynecology, Anhui Provincial Hospital, Anhui Medical University, Hefei, 230001, China.
Department of Obstetrics and Gynecology, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, Anhui, 230001, China.
J Cancer. 2021 Feb 22;12(8):2295-2316. doi: 10.7150/jca.53395. eCollection 2021.
Ovarian clear cell carcinoma (OCCC) is a special pathological type of epithelial ovarian carcinoma (EOC) and has a high prevalence in Asia without specific molecular subtype classification. Endometriosis is a recognized precancerous lesion that carries 3-fold increased risk of OCCC. Ovarian endometrioid carcinoma, which also originates from endometriosis, shares several features with OCCC, including platinum resistance and younger age at diagnosis. Patients with OCCC have about a 2.5 to 4 times greater risk of having a venous thromboembolism (VTE) compared with other EOC, and OCCC tends to metastasize through lymphatic vesicular and peritoneal spread as opposed to hematogenous metastasis. There is only mild elevation of the conventional biomarker CA125. Staging surgery or optimal cytoreduction combined with chemotherapy is a common therapeutic strategy for OCCC. However, platinum resistance commonly portends a poor prognosis, so novel treatments are urgently needed. Targeted therapy and immunotherapy are currently being studied, including PARP, EZH2, and ATR inhibitors combined with the synthetic lethality of ARID1A-dificiency, and MAPK/PI3K/HER2, VEGF/bFGF/PDGF, HNF1β, and PD-1/PD-L1 inhibitors. Advanced stage, suboptimal cytoreduction, platinum resistance, lymph node metastasis, and VTE are major prognostic predictors for OCCC. We focus on update pathogenesis, diagnostic methods and therapeutic approaches to provide future directions for clinical diagnosis and treatment of OCCC.
卵巢透明细胞癌（OCCC）是上皮性卵巢癌（EOC）的一种特殊病理类型，在亚洲发病率较高，且没有特定的分子亚型分类。子宫内膜异位症是一种公认的癌前病变，患OCCC的风险增加3倍。同样起源于子宫内膜异位症的卵巢子宫内膜样癌与OCCC有一些共同特征，包括铂耐药和诊断时年龄较轻。与其他EOC相比，OCCC患者发生静脉血栓栓塞（VTE）的风险大约高2.5至4倍，并且OCCC倾向于通过淋巴管和腹膜扩散转移，而非血行转移。传统生物标志物CA125仅有轻度升高。分期手术或最佳细胞减灭术联合化疗是OCCC常见的治疗策略。然而，铂耐药通常预示预后不良，因此迫切需要新的治疗方法。目前正在研究靶向治疗和免疫治疗，包括PARP、EZH2和ATR抑制剂联合ARID1A缺陷的合成致死性，以及MAPK/PI3K/HER2、VEGF/bFGF/PDGF、HNF1β和PD-1/PD-L1抑制剂。晚期、次优细胞减灭术、铂耐药、淋巴结转移和VTE是OCCC的主要预后预测因素。我们着重介绍最新的发病机制、诊断方法和治疗方法，为OCCC的临床诊断和治疗提供未来方向。
