Introduction: Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with aggressive behavior. Most of our knowledge about this rare tumor is based on retrospective case series. This study aimed at analyzing the clinicopathological features and outcomes of patients treated at a tertiary cancer center in India.
Patients and methods: We retrospectively reviewed the data of patients with ACC registered from January 2006 to December 2015.
Results: Thirty-seven patients were included in the study, 20 males and 17 females. Median age was 49 (18-78) years. Hormonal overproduction was noticed in 27% of patients. Median tumor size was 10 cm (2-22). Seventeen patients had metastatic disease and 20 patients were localised at diagnosis. Median follow-up was 22 months and median overall survival (OS) was 23.46 months. OS at 2 years and 5 years was 46.1% and 21%, respectively. The median disease-free survival (DFS) was 20 months. DFS at 2 years and 5 years was 45% and 24%, respectively. Age, sex, tumor size, hormonal overproduction, tumor laterality, and stage of the disease did not influence survival. However, advanced stage was associated with higher risk for recurrence. (P = 0.03).
Conclusion: ACC is a rare endocrine malignancy with very poor survival rates. Rate of recurrence is high even after complete surgery. Systemic treatment options are limited. Newer agents are needed to improve outcome.