Introduction: Advanced adrenocortical carcinoma has a poor prognosis and is treated with chemotherapy that includes mitotane with etoposide, doxorubicin, and cisplatin as first-line therapy. However, second-line therapy has not been determined yet. Pembrolizumab has been approved for high microsatellite instability for which standard treatments have failed.
Case presentation: Here, we present a patient with advanced adrenocortical carcinoma treated with complete surgical resection. 21 months later, he had local and metastatic recurrences. After four cycles of first-line therapy, we switched to pembrolizumab because microsatellite instability-high was detected in his tumor. He has received mitotane and pembrolizumab for 15 months, and this has exerted a radiographical response without severe adverse events.
Conclusion: We presented a patient with microsatellite instability-high advanced adrenocortical carcinoma treated with pembrolizumab and mitotane.
Keywords: adrenocortical carcinoma; microsatellite instability; mitotane; pembrolizumab; tumor‐agnostic treatment.
© 2023 The Authors. IJU Case Reports published by John Wiley & Sons Australia, Ltd on behalf of Japanese Urological Association.