Pathogenesis of adrenocortical incidentalomas and genetic syndromes associated with adrenocortical neoplasms.
The study of genetic syndromes associated with adrenocortical tumors (Beckwith-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 found 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-independent manner when various membrane receptors of the seven transmembrane superfamily are "illegitimately" expressed.
Gicquel
C
C
Laboratoire d'Explorations Fonctionnelles Endocriniennes, Hôpital Trousseau, Paris, France.
Bertherat
J
J
Le Bouc
Y
Y
Bertagna
X
X
D000236:Adenoma / Q000175:diagnosis / Q000235:genetics*; D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis / Q000235:genetics*; D002880:Chromosomes, Human, Pair 11; D016147:Genes, Tumor Suppressor; D006133:Growth Substances / Q000235:genetics; D006801:Humans; D009154:Mutation; D009857:Oncogenes; D015398:Signal Transduction / Q000235:genetics
D006133:Growth Substances