Surgery is associated with improved survival for adrenocortical cancer, even in metastatic disease

Abstract

Background: Adrenocortical carcinoma (ACC) is a rare but lethal tumor. Predictors of survival include earlier stage at presentation and complete operative resection. We assessed effect of treatment and demographic variables on survival.

Methods: ACC cases were abstracted from the California Cancer Registry and Office of Statewide Health Planning and Development (1999-2008). Predictors included patient demographics, comorbidities, tumor size, stage, and treatment (none, surgery, chemotherapy and/or radiation [CRT], and surgery plus CRT).

Results: We studied 367 patients with median tumor size of 10 cm. At presentation, 37% had localized, 17% had regional, and 46% had metastatic disease. Median survival was 1.7 years (7.4 years local, 2.6 years regional, and 0.3 years metastatic, P < .0001). One-year and 5-year survival was: 92%/62% (local); 73%/39% (regional); and 24%/7% (metastatic). Increased age (hazard ratio [HR] 1.16) and Cushing's syndrome (HR 1.66) worsened survival (P < .05). Low socioeconomic status worsened survival in local and regional disease (P < .05). In multivariable regression, both surgery (regional HR 0.13; metastatic HR 0.52) and surgery plus CRT (regional HR 0.15; metastatic HR 0.31) improved survival compared with no treatment (P < .02).

Conclusion: In ACC, surgery is associated with improved survival, even in metastatic disease. Surgery should be considered for select patients as part of multimodality treatment.