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Endocrine Abstracts (2009) 20 S23.1

Medizinische Klinik Innenstadt, München, Germany.


Tumors of the adrenal cortex can present as incidental findings during abdominal imaging, as the cause of steroid excess and/or as malignancy. The most common adrenal disorder encountered today is the adrenal incidentaloma, which is mostly benign but can be associated with (subclinical) Cushing’s syndrome or primary aldosteronism. In contrast, adrenocortical carcinoma (ACC) represents a rare but highly malignant tumor entity. Over the last years studies including expression profiling of tissue samples, in vitro examination of tumor related pathways and genetic examination of patient cohorts with specific adrenal disorders have uncovered a number of mechanisms relevant for the molecular pathogenesis of adrenocortical tumors. Furthermore, hereditary diseases including Li-Fraumeni and Beckwith-Wiedeman syndrome have allowed important insights in mechanisms of adrenal tumorigenesis. Finally, during the last decade a number of mouse systems have been developed with distinct features of adrenal tumorigenesis that have aided as in vivo models. IGF-2 is considered one of the most potent growth factor for the adrenal. Accordingly, genetic studies and expression profiling on adrenocortical carcinomas have demonstrated a variety of genetic alterations resulting in IGF-2 overexpression. Conversely, loss of peptide hormone expression such as the Bone morphogenetic proteins (BMPs) also have been demonstrated to impact growth and function of ACCs. Furthermore, the Wnt/β-catenin pathway has been recently suggested to be activated in both benign and malignant adrenocortical tumors. Activating mutations of the β-catenin gene were found with similar frequencies in adrenal adenomas and carcinomas whereas abnormal localization of β-catenin was observed at a higher rate in adenomas than in carcinomas. In addition, somatic mutations in the regulatory R1A subunit of protein kinase A (PRKAR1A), which is a key component of the cAMP signaling pathway that has been implicated in endocrine tumorigenesis have been demonstrated in sporadic secreting adrenocortical adenomas. Taken together, a number of molecular mechanisms have been recently identified that contribute to adrenocortical growth and function. Future challenges will include translation of these molecular advances into clinical practice to improve diagnosis and treatment of patients with adrenocortical tumors.

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