Background. Current American Joint Committee on Cancer/International Union against Cancer (AJCC/UICC) and European Network for the Study of Adrenal Tumors staging for adrenocortical carcinoma (ACC) have not shown a survival difference between patients with stage I/II disease. This study evaluates current staging systems for survival prediction using a larger cohort and assesses whether incorporating age into ACC staging improves survival predictions. Methods. Patients in the National Cancer Data Base (1985-2006) with a diagnosis of ACC were identified and staged using a novel TNM-A staging system: Stage I (T1/T2N0M0, age #55), stage II (T1/T2N0M0, age >55), stage III (T1/T2N1M0 or T3/T4N0-N1M0, any age), or stage IV (any T any NM1, any age). Differences in overall survival (OS) by stage were compared using a Cox proportional hazards model. Results. Staging was derived for 1,579 of 3,262 patients. Median age was 54 years; mean tumor size was 11.6 cm. Using current staging, differences in 5-year OS was observed only between patients with stages II/III and III/IV ACC. With TNM-A staging, differences in 5-year OS between all stages was significant (I/II [P < .003], II/III [P < .0001], III/IV [P < .0001]). Conclusion. A staging system that incorporates patient age better predicts 5-year OS among patients with stages I/II ACC. Consideration should be given to including age in staging for ACC, because it may better inform providers about treatment and prognosis.
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