Trends in the incidence of adrenocortical carcinoma and pheochromocytoma/paraganglioma in Taiwan

Abstract

Background Adrenal malignancies are rare endocrine tumors. Recent epidemiological studies suggest that incidence rates of adrenal malignancies are increased in certain countries. In this study, we aimed to analyze temporal trends in the incidence of adrenocortical carcinoma and pheochromocytoma/paraganglioma (PPGL). Methods Cases of adrenal malignancies were identified from the Taiwan cancer registry, and age-standardized incidence rates (ASRs) were calculated. Annual percent change (APC) or average APC was obtained using joinpoint regression models. Results The ASRs of adrenocortical carcinoma ranged from 0.021 to 0.135 per 100 000 person-years. The average APC between 1995 and 2020 was 6.633% (95% confidence interval, 2.740 to 9.403). The ASRs of PPGL ranged from 0.005 to 0.056 per 100 000 person-years before 2017 and, because of changes in diagnostic criteria, the median ASR of PPGL rose to 0.314 per 100 000 person-years after 2017. The APC between 1996 and 2016 was 3.554% (95% confidence interval, -1.103 to 8.264). Conclusions The annual incidence of adrenocortical carcinoma has significantly increased in Taiwan over the past two decades. The incidence of malignant PPGL remained stable over this time period.