Only 3 gene mutations were found among 509 genes examined in the ACTH-producing PC in our study, including mutations in ATRX...and P53. In addition, we uniquely identified the novel mutation in PTEN...by comparison analysis with published data on PCs. The patient's residual tumor was well-controlled by temozolomide and general radiotherapy for 3 months, and there have been no new lesions or metastases.