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Adrenocortical carcinoma (ACC), particularly when occurring during childhood, has been a traditional component of the tumor spectrum of Li-Fraumeni syndrome. Recent research has defined a significant risk increase of ACC with other familial cancer syndromes, such as Lynch syndrome and multiple endocrine neoplasia. ACC patients can serve as index patients for a new family diagnosis of a hereditary syndrome, allowing for further family cascade genetic testing, impacting the care and surveillance for patients and at risk family members. Individuals carrying pathogenic genetic variants can embark on a regular preventive screening and surveillance protocol likely reducing morbidity and mortality. Although several of these hereditary predisposition syndromes lead to a very high relative risk increase for ACC, the absolute risk most often does not reach a level to recommend general screening for ACC in carriers of pathogenic mutations. The larger value lies in the ability to screen for other commonly associated tumors in pathogenic variant carriers, such as colon cancer with Lynch syndrome. Here, we review the risk for ACC associated with hereditary syndromes and suggest an approach for genetic evaluation for ACC patients.Le texte complet de cet article est disponible en PDF.