Abstract
Background: Advanced stages of ovarian clear cell carcinoma have poorer prognoses than many other ovarian cancers. When standard treatments are ineffective, alternative options are needed. Case: A 56-year-old woman was diagnosed with ovarian clear cell carcinoma and treated with surgical management as well as carboplatin and paclitaxel. At recurrence, her disease progressed despite multiple chemotherapy regimens. A durable response was achieved first with alpelisib, chosen based on genomic testing. When progression occurred on this agent, a partial disease response was achieved with the combination of nivolumab and ipilimumab. Conclusion: The use of targeted therapies as well as the combination of nivolumab and ipilimumab is a promising option in advanced and recurrent cases of ovarian clear cell carcinoma.