Endocrine tumor syndromes constitute a group of disorders characterized by tumors in hormone-producing tissues. These conditions predominantly affect younger patients and often have a familial inheritance. Advances in molecular genetics in recent decades have facilitated the identification of several genes associated with these tumors. The recent World Health Organization classification of adrenocortical tumors integrates the latest developments in pathology, oncology, and molecular biology. In addition, this updated classification includes adrenal cortical diseases based on an understanding of germline susceptibility to these conditions and their clonal-neoplastic nature. Catecholamine-secreting tumors, including pheochromocytoma and paraganglioma, have been found to have a genetic predisposition in as many as 80% of cases. Compared to sporadic cases, endocrine tumor syndromes are more likely to present bilaterally and show synchronous or metachronous disease. This highlights the critical need for early diagnosis, intervention, and ongoing surveillance. This review focuses on the clinical manifestations and genetic basis of endocrine tumor syndromes originating from the adrenal glands.
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