Pathogenesis of adrenocortical incidentalomas and genetic syndromes associated with adrenocortical neoplasms

The study of genetic syndromes associated with adrenocortical tumors (Beckw ith-Wiedemann, Li-Fraumeni, McCune-Albright, Carney, and multiple endocrine neoplasia type 1) has shed light on the molecular basis of tumorigenesis. Abnormalities at the 11p15 locus appear as crucial and frequent events foun d specifically in malignant, sporadic tumors, leading to overexpression of a growth-promoting factor and loss of expression of tumor suppressor genes. In benign tumors, the cAMP pathway can be exacerbated in an ACTH-independe nt manner when various membrane receptors of the seven transmembrane superf amily are "illegitimately" expressed.