Neuroendocrine carcinomas are ubiquitous neoplasms that may occur anywhere in the human body. A unifying concept regarding the classification of these tumors has been controversial. Although most neuroendocrine neoplasms occur in the gastrointestinal tract, current concepts regarding classification and nomenclature are being driven by studies of thoracic tumors. One issue that has been put forward to keep separate nomenclatures for these tumors in different organ systems is the different clinical behavior of these neoplasms in different systems. The most important aspect regarding this group of tumors is the fact that they should be considered neoplasms capable of local recurrence and distant metastasis. Close clinical correlation and appropriate treatment are important to improve the survival rate in this group of patients.