Neuroendocrine carcinomas (NECs) form a diverse group of malignant epithelial neuroendocrine neoplasms, ranging from well-differentiated to high-grade tumors. The classification of neuroendocrine tumors has evolved dynamically in different directions; tumors of the gastrointestinal tract, pancreas, head and neck, skin, and lungs have been described. The latter is important, as many clinicians use the World Health Organization (WHO) pulmonary guidelines to classify extrapulmonary NECs. The WHO currently recognizes four major types of lung neuroendocrine tumor based on the mitotic rate and extent of necrosis: typical and atypical carcinoids, and small and large cell NECs. The subtype is of prognostic and therapeutic significance.
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