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Diagnostic and Therapeutic Strategies in Endocrine Duodenopancreatic Tumours

Endocrine tumours have many similar features whether they are located in the pancreas, in the duodenum, or in the ampulla. They are either sporadic or genetic, solitary or multiple, well differentiated or poorly differentiated, with a common potential of lymph node and metastatic extension. The qualifying of endocrine has replaced the other previous terminologies : neuroendocrine, APUDoma, carcinoid. Most endocrine duodeno-pancreatic tumours EDPT are sporadic but some of them occur in a genetic context such as MEN type 1, von HippelLindau disease, Recklinghausen disease. The genetic context may be obvious or latent. Consequently, due to the utmost importance of this characteristic, a particular attention must be paid in each patient to the familial survey and to the clinical and biological investigations for the detection of a genetic syndrome. These tumours may be functional (inducing clinical symptoms due to an abnormal peptidic secretion) or non functional (without any specific symptom related to any abnormal hypersecretion). Therapeutic strategies vary according to the differentiation of tumoural cells, undifferentiated tumours being most of the times highly aggressive and relevant to oncological therapy.