Gangliocytic paraganglioma(GP) is rare neuroendocrine tumor(NET) with a good prognosis that commonly arising from duodenum. Although the tumor is characterized by its unique triphasic cells(epithelioid, spindle, and ganglion-like cells), the proportions of these three tumor cells vary widely from case to case,and occasionally, morphological and immunohistochemical similarities are found between GP and NET G1(carcinoid tumors). Further, GP accounts for a substantial number of duodenal NETs. Therefore, GP continues to be misdiagnosed, most often as NET G1. However, GP has a better prognosis than NET G1, and it is important to differentiate GP from NET G1. In this article, I wish to provide up-to-date clinicopathological information to help oncologists gain better insight into the diagnosis and clinical management of this tumor.
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