Ovarian clear cell carcinoma

In the United States, it is estimated that there will be 21,410 new diagnoses of ovarian cancer, and 13,770 deaths from ovarian cancer. Ovarian clear cell carcinoma represents 5% to 10% of ovarian cancers in the United States. Ovarian clear cell carcinoma is a rare ovarian malignancy; as a result, clinical trials are not powered to make conclusions for ovarian clear cell carcinoma despite known differences in response to treatment. Recommendations for surveillance and treatment of ovarian clear cell carcinoma are nonspecific and follow recommendations for the most common histology of high-grade serous ovarian cancer. In this chapter, we will highlight the known distinct features of ovarian clear cell carcinoma and treatment recommendations.