Prognostic factors and treatment outcomes in patients with adrenal cortical carcinoma: A registry-based study

Adrenal cortical carcinoma (ACC) is rare in clinical practice, and its prognosis and treatment have not been systematically studied. This study aimed to investigate the prognostic factors and impact of treatment strategies on overall survival (OS) and cancer-specific survival (CSS) in patients with ACC, using Surveillance, Epidemiology, and End Results registry data. Patient's data for ACC pathologically diagnosed between 2000 and 2021 were identified from Surveillance, Epidemiology, and End Results database. The Kaplan-Meier method was used to assess OS and CSS. While Cox proportional hazards regression analysis was used to evaluate the risk predictors of OS and CSS, the effects of surgery, chemotherapy, and radiotherapy on OS and CSS were also assessed using directed acyclic graphs-guided multivariate Cox regression model. A total of 444 patients with ACC were enrolled. The median OS and CSS in this cohort were 25 and 30 months, respectively. The OS at 1, 2, and 5 years were 62.7%, 50.2%, and 31.5%, respectively, while the CSS at 1, 2, and 5 years were 66.6%, 54.8%, and 37.2%, respectively. Patients with ≥ 53 years old, pathological grade Ⅲ/Ⅳ, clinical stage Ⅲ/Ⅳ, T3/4, N1, M1, and none receiving the therapy of surgery or radiation had worse survival. Old age, worse clinical stages, poor pathological grade, distant metastasis, no surgical treatment, and no radiation therapy were risk predictors of OS and CSS. In addition, receiving surgery or radiation therapy improved OS and CSS in the directed acyclic graph-guided multivariate Cox regression model. ACC has malignant behavior and a relatively poor prognosis. Old age, poor pathological grades, late clinical stages, and advanced tumor node metastasis classification indicate poor survival. While surgical resection of tumor lesions is one of the effective treatments, which can improve the outcome of patients with ACC, radiation therapy may be a palliative and beneficial treatment for patients with advanced disease.