Improved Survival in Patients with Stage II Adrenocortical Carcinoma Followed Up Prospectively by Specialized Centers

Context: Median survival in stage II adrenocortical carcinoma (ACC) differs widely in published series ranging between 23 and more than 60 months. We hypothesized that these results may have been affected by a referral bias because many patients may contact specialized centers only after recurrence. Objective: The objective of the study was a comparison of outcome in patients with stage II ACC who were followed up prospectively early after surgery and were counseled by a specialized center (prospective group) with patients who registered with the German ACC registry later than 4 months after diagnosis (retrospective group). Patients/Methods: The study was a cohort analysis in 149 adult patients with stage II ACC. Results: Patients who were followed up prospectively (n = 30) had a lower recurrence rate and a superior 5-yr survival compared with the 119 patients in the retrospective group (30 vs. 74%, P < 0.01 and 96 vs. 55%, P < 0.05, respectively). In the retrospective group, 67% of the patients had registered only after disease recurrence. In the remaining patients, the recurrence rate was low (21%), and the 5-yr survival was greater than 95%. More patients in the prospective group received adjuvant mitotane (53 vs. 16%, P < 0.001), and adjuvant mitotane was associated with improved survival [hazard risk 0.35 (95% confidence interval 0.13-0.97); P = 0.04]. However, the survival advantage was maintained when only patients without mitotane therapy were analyzed. Conclusions: Patients who are followed up prospectively after surgery for stage II ACC and receive early specialized care have a much better prognosis than previously reported due to a major referral bias in previous series and use of adjuvant mitotane. These findings will impact on the perception of prognosis in newly diagnosed stage II ACC. (J Clin Endocrinol Metab 95: 4925-4932, 2010)