April - June 2010, Volume 6, Issue 2

Bertagna X

Adrenal cortical carcinoma: recent advances in diagnosis and clinical management

Acta Endo (Buc) 2010, 6 (2): 237-250
doi: 10.4183/aeb.2010.237

This is a rare disease, which is often diagnosed at a late stage, and which has no fully efficacious medical treatment; hence its dismal prognosis. Yet its clinical presentation is changing (more and more as an "incidentaloma"), new insights have emerged on its\r\npathophysiological mechanisms (through the genetic elucidation of familial syndromes), and Reference Centers within National and European Networks are now offering more\r\nstandardized and efficient multidisciplinary clinical management to these difficult patients. There are three important messages:\r\n-The single best likelihood of "cure" is when a localized tumor can be subjected to\r\n"complete" surgical removal. An early diagnosis is crucial. In front of an adrenal tumor, an endocrinologist must always ask two questions: could it be a pheochromocytoma? Could it be an adrenal cortical carcinoma? In both cases, it can be a vital question which can make the difference between a fatal issue or cure! A thorough biological and imaging work-up is mandatory for a proper diagnosis. An expert endocrinologist will recognize a suspicious\r\nadrenal "incidentaloma", diagnose steroid hypersecretion, suspect a familial disease, communicate with the pathologist and surgeon, and, when necessary, manipulate difficult drugs like O,p' DDD, participate in multidisciplinary discussions with oncologists,\r\nradiotherapists, molecular biologists?.\r\n-Research, both basic and clinical, is key to further progress: a better understanding of the biology of these tumors has already shed some light on the role of signaling pathways, on some familial syndromes, on new prognostic markers. There is some hope that these approaches will provide us with targeted therapies. Alternatively, progress in our understanding on the general mechanisms of tumor growth might help us design new therapeutic tools using antiangiogenic agents and/or immunotherapy.\r\n-Reference Centers and National (and European) Networks are essential to optimize individual patient management, as well as to organize basic research and multicenter clinical\r\ntrials in this rare disease. For an individual patient, difficult therapeutical options are best offered by a multidisciplinary team (endocrinologist, oncologist, surgeon, radiologist, pathologist, radiotherapist) In order to boost scientific exchange, to facilitate and harmonize\r\nanalyses of biological samples, to allow the design of epidemiologic studies or prospective therapeutic trials, several European countries have developed their national networks dedicated to the study of adrenal tumors: COMETE in France, NISGAT in Italy,\r\nGANIMED in Germany. These national networks have merged into the European network ENS@T (European Network for the Study of Adrenal Tumors): its goal is to create a\r\nnetwork large enough to allow the recruitment of patients with rare diseases to harmonize diagnostic and therapeutic procedures at the European level.

Keywords: adrenal, mitotane, molecular markers, cancer

Correspondence: Xavier Bertagna, AP-HP, Cochin Hospital,Endocrine and Metabolic Disease Department;Reference Center for Rare Adrenal Cancers INCa-COMETE Endocrinology Department, Cochin Institute, INSERM U-1016 Medecine Faculty Paris Descartes, 27, rue du Faubourg Saint Jacques Paris 75679-cedex 14, FRANCE;E-mail: xavier.bertagna@cch.aphp.fr