Table 2.

Characteristics of ovarian clear cell carcinomas and potential molecular targets

Clinical features	Mutated genes and overexpressed proteins	Reference
Higher incidence among Asian women, particularly Japanese women	ARID1A is mutated in 46 % of CCC patients (loss of function)	[62, 85]
Clear cell carcinoma has a strong association with endometriosis	PIK3CA is mutated in 33 % of CCC patients (activation mutation)	[53]
Slow tumor growth, facilitating early detection	Annexin A4 is expressed in almost all CCC patients	[20, 22]
Strong resistance to platinum-based chemotherapy	mTOR is overexpressed in 80 % of CCC patients	[63]
Promising regimens with favorable and stable response for ovarian clear cell carcinoma have remained elusive	HNF-1β is expressed in almost all CCC patients	[52, 64, 71–73]
Low frequency of BRCA1/2 mutations	ZNF217 is overexpressed in 20 % of CCC patients	[65]
Low frequency of p53 mutations (15 %)	VEGF is strongly expressed in both early and advanced stages of CCC	[66]
	EGFR is overexpressed in 60 % of CCC patients	[59]
	MET is overexpressed in approximately 20 % of CCC patients	[85]
	HER2 is overexpressed in 14–42.9 % of CCC patients	[52, 53]
	PPMID expression is observed in 10 % of CCC patients	[68]
	PPP2R1A is overexpressed in 7 % of CCC patients	[52]
	KRAS is overexpressed in 5 % of CCC patients	[52]

ARID1A AT-rich interactive domain 1A (SWI-like) gene, BRCA 1/2 breast cancer susceptibility gene 1/2, EGFR epidermal growth factor receptor, HER2 human epidermal growth factor receptor 2, HNF-1β hepatocyte nuclear factor 1β, mTOR mammalian target of rapamycin, PIK3CA phosphoinositide 3-kinase catalytic-α, PPMID protein phosphatase magnesium-dependent, PP2R1A protein phosphatase 2, regulatory subunit A, VEGF vascular endothelial growth factor, ZNF217 zinc finger protein 217