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Neuroendocrine neoplasm (NEN) is a group of heterogeneous malignancies arising from neuroendocrine cells and peptidergic neurons. According to its cell differentiation, NEN can be divided into two distinct groups as well-differentiated neuroendocrine tumor (NET) and poorly-differentiated neuroendocrine carcinoma (NEC). For advanced NEN, medication is the preferred treatment. In this article, we reviewed current clinical trials in medical treatment for NEN and aimed to provide treatment reference in clinical practice.