[Endocrine tumors: biology and physiopathology]

Endocrine tumors are defined as neoplastic lesions resulting from the proliferation of cells engaged in an endocrine differentiation pathway, as shown by their expression of a set of specific markers, including true endocrine markers (such as chromogranine A) and neuro-endocrine markers, shared between neurons and endocrine cells (such as synaptophysin). The demonstration of the synthesis and secretion of one or several hormones is not necessary for the assessment of the endocrine nature of a tumor; only tumors associated with a clinical syndrome resulting from hormone overproduction can be said functioning endocrine tumors. Beyond their common features, endocrine tumors are characterized by a marked diversity, which results from the large functional, structural and embryological heterogeneity of normal endocrine cells. The natural history of endocrine tumors is also characterized by a marked heterogeneity in their evolution and rate of progression. While most endocrine tumors are locally and slowly evolving, some of them behave as truly malignant tumors, as shown by their capacity of metastatic dissemination and their fatal evolution. A better understanding of the cellular and molecular mechanisms involved in tumor progression and metastatic dissemination is necessary for the identification of new prognostic tools and novel therapeutic targets.