Biological and molecular aspects of gastroenteropancreatic neuroendocrine tumors

Neuroendocrine tumors of the digestive tract are rare entities characterized by significant phenotype differences and traditionally considered to originate from cells of the diffuse endocrine system of the pancreas and gut. Two major categories with significant phenotype and clinical behavior differences are identified as well-differentiated and poorly differentiated tumors. Investigation on the molecular basis of tumor development points to an important role for the multiple endocrine neoplasia syndrome type-1 (MEN1) gene because of its frequent abnormality observed both in well-differentiated and poorly differentiated tumors. Other genes are possibly involved, though the available data need support from studies on larger series of tumors.