The Role of Surgery in the Management of Recurrent Adrenocortical Carcinoma

Context: Surgery is the standard of care for localized adrenocortical carcinomas, but its role for recurrent disease is not well defined. Objective: Our objective was to evaluate clinical outcome after surgery for recurrence. Design: We conducted a retrospective analysis in 154 patients with first recurrence after initial radical resection from the German Adrenocortical Carcinoma Registry. Main Outcome Measures: We evaluated progression-free survival (PFS) and overall survival (OS) by Kaplan-Meier method and identified prognostic factors by Cox regression analysis. Result: Atotal of 101 patients underwent repeated surgery (radical resection, n = 78), and 99 received (additional) nonsurgical therapy. After a median of 6 (1-221) months, 144 patients (94%) experienced progression. Multivariate analysis adjusted for age, sex, tumor burden, time to first recurrence (TTFR), surgery for recurrence (including resection status), and additional therapy indicated that only two factors were significantly associated with shorter PFS [hazard ratio for progression: for TTFR <= 12 months, 1.8 (95% confidence interval <= 1.3-2.6) vs. TTFR <= 12 months; for macroscopically incomplete resection, 3.4 (1.5-7.9), and fornosurgery, 3.4 (1.6-7.0) vs. microscopically complete (R0)-resection and OS [hazard ratio for death: for TTFR <= 12 months, 3.1 (2.0-4.7) vs. TTFR <= 12 months; for macroscopically incomplete resection, 2.7 (1.1-6.9), and no surgery, 4.2 (1.8-9.6) vs. R0-resection]. Patients who had both TTFR over 12 months and R0-resection of recurrent tumors (n = 22) had the best prognosis (median PFS, 24 months; median OS, >60 months). Conclusions: The best predictors of prolonged survival after first recurrence are TTFR over 12 months and R0-resection. Our data suggest that patients with longer TTFR and tumors amenable to radical resection should be operated, where as individualized treatment decisions are needed for patients with short TTFR or with not completely resectable tumors. (J Clin Endocrinol Metab 98: 181-191, 2013)