Gangliocytic paraganglioma: An overview and future perspective

Abstract

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.

Keywords: Neuroendocrine tumor, Gangliocytic paraganglioma, Progesterone receptor, Pancreatic polypeptide, Literature survey

Core tip: Although gangliocytic paraganglioma (GP) has been regarded as a rare neuroendocrine tumor (NET), GP accounts for a significant number of duodenal NETs. Morphological and immunohistochemical similarities between GP and NET G1 often lead to misdiagnoses of both. However, the prognosis is often better for patients with GP than for those with NET G1. Therefore, it is important to differentiate GP from NET G1. This editorial provides up-to-date data on the clinicopathological characteristics of GP and emphasizes the importance of confirming progesterone receptor and pancreatic polypeptide immunoreactivity for differentiating GP from NET G1.