Abstract
Adrenocortical carcinomas are rare malignant tumors. However, the high incidence of the adrenocortical tumors in pediatric and adult patients from the Southern region of Brazil is particularly remarkable, since it has been estimated to be approximately ten times greater than the worldwide incidence. Adrenocortical tumors occur as a component of several hereditary tumor syndromes, which include the Li–Fraumeni syndrome, Beckwith–Wiedemann syndrome, multiple endocrine neoplasia 1, Carney complex, and congenital adrenal hyperplasia. The study of these rare genetic syndromes has greatly contributed to the elucidation of sporadic adrenocortical tumorigenesis. This chapter summarizes the molecular alterations likely involved in the multistage tumorigenesis of familial or sporadic adrenocortical adenomas and carcinomas.
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