Clinicopathological Features, Treatment Outcomes, and Prognostic Factors in Adrenocortical Carcinoma: A Single-Center Experience

Abstract

gland is adrenocortical carcinoma (ACC). ACC is a highly rare malignancy, with an incidence of 0.7-2 per million population.1 Although a diagnosis can be made at any age, ACC more frequently affects women, and its incidence peaks in the fourth to fifth decades of life.2 ACCs are generally sporadic; however, they are occasionally observed as a component of hereditary syndromes like Li-Fraumeni or Beckwith-Wiedemann syndrome.3 Approximately 60% of patients present with symptoms owing to adrenal hormone secretion, where cortisol excess (Cushing syndrome) is the leading manifestation followed by secretion of sex hormones (primarily androgen).1,4 Non-functional ACCs cause symptoms owing to tumor burden, particularly abdominal pain and weight loss. Most ACC cases are diagnosed at an advanced stage, and 5-year survival remains below 50%.5