Hereditary Conditions Associated with Adrenocortical Carcinoma, Pheochromocytoma, and Other Adrenal Tumors: Genetic Testing and Management Recommendations.

This article summarizes hereditary conditions associated with adrenal tumors, emphasizing the importance of germline genetic testing in patients with adrenocortical carcinoma (ACC) and pheochromocytoma/paraganglioma (PPGL). ACC is strongly linked to syndromes such as Li-Fraumeni, Lynch, and Beckwith-Wiedemann, while PPGL has one of the highest hereditary rates among endocrine tumors, often involving SDHx genes and other susceptibility genes, including VHL, RET, NF1, MAX, and TMEM127. The article outlines clinical features, gene-specific risks, management considerations, and evolving surveillance guidelines. Identifying hereditary predispositions improves patient care, guides targeted surveillance, and allows cascade testing for at-risk family members.