ABSTRACT
The diffuse neuroendocrine system is divided into central and peripheral divisions: the first contains the cells of the hypothalamopituitary axis and the pineal gland, whereas the cells of the second division are primarily located in the gastrointestinal tract and pancreas, where they comprise the gastro-entero-pancreatic endocrine cells. Screening in a large series of samples from different types of endocrine and non-endocrine cancers revealed that neither germline nor somatic aryl hydrocarbon receptor–interacting protein gene mutations are associated with these neoplasms. Neuroendocrine neoplasms represent a heterogeneous group with respect to their response to cytotoxic agents, which is site specific. Diagnostic tests include the endocrine demonstration of non-suppressible and excessive levels of circulating adrenal steroids and appropriate diagnostic imaging tests. Between one-quarter and one-third of patients with primary adrenal carcinoma have clinical evidence of endocrine dysfunction at presentation, most commonly Cushing’s syndrome, often supplemented by virilism.
