Neuroendocrine neoplasm of the larynx is a morphologically heterogeneous group of tumors. This unusual neoplasm includes the four different type of tumors. Paraganglioma is the best-known neural origin tumor. The epithelial origin tumor is further divided based on the degree of differentiation into well, moderately, and poorly differentiated carcinoma. The diverse biological behavior of neuroendocrine neoplasm of larynx makes an accurate diagnosis of paramount importance, since treatment depends on diagnostic accuracy. The diagnosis is based primarily on light microscopy, and immunohistochemical and/or ultrastructural investigations are needed to diagnose correctly. The mainstay of treatment of moderately differentiated neuroendocrine carcinoma of the larynx is surgery. Elective neck treatment should be performed considering the high likelihood of cervical lymph node metastases. The 5-yr survival rate approximates 50%, and 10-yr survival rate is only 30%
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