Analysis of clinical and pathological characteristics of classic adrenocortical carcinoma

Adrenocortical carcinoma (ACC) is a rare malignancy; however, its specific characteristics within the Chinese population remain poorly characterized. This study investigates the clinical and pathological characteristics of classic adrenocortical carcinoma in a Chinese cohort to improve understanding of the disease and inform management strategies. We retrospectively investigated the clinical and pathological features of 138 classic ACC patients treated at Peking Union Medical College Hospital from January, 1984 to January, 2024. The gene expression profile of 79 ACC patients with survival data was downloaded from the TCGA database. P < 0.05 was considered statistically significant. We found that classic ACC was more common in women than in men, and the majority of classic ACC patients were diagnosed over 40. The tumor exhibited a slight right-sided laterality, with the incidence on the right side being 1.28 times that of the left. The median tumor diameter was 9.05 cm at diagnosis. We revealed that the Ki-67 index of most patients was over than 5% and the median of Ki-67 index was 20%. The majority of classic ACC patients were categorized into ENSAT stage II (47.1%) and III (27.54%) at diagnosis. In addition, classic ACC patients with advanced stage tended to get higher levels of Ki-67 index (P = 0.002). Patients with higher Ki-67 index (P = 0.036) and Ki-67 gene expression (P < 0.001) had worse overall survival rate. Pathway analysis in patients with high MKI67 expression revealed an enrichment of genes associated with cell cycle regulation and p53 signaling, suggesting a potential link between high proliferative activity and these molecular pathways. In conclusion, this study provides valuable insights into the clinical and pathological profiles of ACC in the Chinese population, facilitating better diagnostic approaches.