IntroductionAdrenocortical carcinoma (ACC) is a notoriously aggressive cancer with a dismal prognosis, especially for patients with metastatic disease. Metastatic ACC is classically a contraindication to operative management. Here, we evaluate the impact of primary tumor resection and metastasectomy on survival in metastatic ACC.MethodsWe performed a retrospective cohort study of patients with metastatic ACC (2010–2019) utilizing the National Cancer Database. The primary outcome was overall survival (OS). Cox proportional hazard models were developed to evaluate the association between surgical management and survival. Propensity score matching (PSM) was utilized to account for selection bias in receipt of surgery.ResultsOf 976 subjects with metastatic ACC, 38% underwent surgical management. Median OS across all patients was 7.6 months. On multivariable Cox proportional hazards regression, primary tumor resection alone (HR: 0.523; p < 0.001) and primary resection with metastasectomy (HR: 0.372; p < 0.001) were significantly associated with improved OS. Metastasectomy alone had no association with OS (HR: 0.909; p = 0.740). Primary resection with metastasectomy was associated with improved OS over resection of the primary tumor alone (HR: 0.636; p = 0.018). After PSM, resection of the primary tumor alone remained associated with improved OS (HR 0.593; p < 0.001), and metastasectomy alone had no survival benefit (HR 0.709; p = 0.196) compared with nonoperative management; combined resection was associated with improved OS over primary tumor resection alone (HR 0.575, p = 0.008).ConclusionIn metastatic ACC, patients may benefit from primary tumor resection alone or in combination with metastasectomy; however, further research is required to facilitate appropriate patient selection.