Response to mitotane predicts outcome in patients with recurrent adrenal cortical carcinoma

Background: Adrenal cortical carcinoma (ACC) is a rare disease in which recurrence after surgery is common. Mitotane is the primary systemic treatment for recurrent ACC; data are limited regarding the impact of mitotane on recurrent ACC. Methods: We reviewed the records of all patients who underwent surgery for ACC evaluated at our institution from 1991 to 2006. Results: The median disease-free survival for all 186 patients was 12 months and the median overall survival (OS) was 37 months. Higher stage at presentation (P = .002) and tumor cortisol production (P = .007) were associated with a worse OS. Mitotane was given to 67 evaluable patients with recurrent ACC. A response to mitotane was observed in 13 (19%) of the 67 patients. For patients with stable or responding disease to mitotane, the median OS from date of recurrence was 18 months, compared with 9 months (P = .01) for those who progressed. Conclusions: Patients with recurrent ACC who have stable or responding disease to mitotane have a more favorable prognosis than those who progress. Mitotane should be considered in most patients with recurrent ACC, including as preoperative therapy for those with recurrent disease considered for surgical resection.