Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Oncol. Sep 24, 2019; 10(9): 300-302
Published online Sep 24, 2019. doi: 10.5306/wjco.v10.i9.300
Gangliocytic paraganglioma: An overview and future perspective
Yoichiro Okubo
Yoichiro Okubo, Department of Pathology, Kanagawa Cancer Center, Yokohama, Kanagawa 241-8515, Japan
Author contributions: Okubo Y conceptualized this editorial, integrated the data, and wrote the manuscript.
Supported by JSPS KAKENHI, No. JP17K08713 from the Ministry of Education, Culture, Sports, Science and Technology of Japan; and Kanagawa Prefectural Hospitals Cancer Fund, No. 20182019-Nenndo.
Conflict-of-interest statement: The author has no conflict of interest to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Yoichiro Okubo, MD, PhD, Assistant Professor, Department of Pathology, Kanagawa Cancer Center, 2-3-2 Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515, Japan. yoichiro0207@hotmail.com
Telephone: +81-45-5202222 Fax: +81-45-5202202
Received: March 2, 2019
Peer-review started: March 4, 2019
First decision: August 2, 2019
Revised: August 17, 2019
Accepted: August 21, 2019
Article in press: August 21, 2019
Published online: September 24, 2019

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.