Characterization of the Strasbourg Cohort of Patients With Adrenal Cortex Carcinoma

Adrenal corticosteroid carcinoma (ACC) is a rare tumor of the adrenal cortex, with an estimated prevalence of 0.5 to 2 cases per million inhabitants per year. Two peaks in incidence have been described: during the first decade of life and between 40 and 50 years of age, with a slight female predominance (female-to-male ratio of 1.5). The majority of cases (\>90%) are sporadic, particularly in adults. ACC may be discovered incidentally during an imaging examination performed for another reason (10 to 20% of cases) or in connection with a tumor syndrome (40-60% of cases) or hormonal hypersecretion (40 to 74% of cases). The diagnosis of CCS can be suggested by the combination of morphological characteristics seen on imaging and clinical and biological features (secretory syndrome), but only histopathology allows for a definitive diagnosis. Furthermore, histopathology enables the assessmen