Case Report
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 16, 2017; 5(6): 222-233
Published online Jun 16, 2017. doi: 10.12998/wjcc.v5.i6.222
Duodenal gangliocytic paraganglioma with lymph node metastases: A case report and comparative review of 31 cases
Sahara J Cathcart, Aaron R Sasson, Jessica A Kozel, Jennifer M Oliveto, Quan P Ly
Sahara J Cathcart, Department of Pathology and Microbiology, 985900 Nebraska Medical Center, Omaha, NE 68198-5900, United States
Aaron R Sasson, Department of Surgery, Division of Surgical Oncology, Stony Brook Medicine, Stony Brook, NY 11794, United States
Jessica A Kozel, Midwest Pathology Associates, LLC, Overland Park, KS 66210, United States
Jennifer M Oliveto, Department of Radiology, University of Nebraska Medical Center, Omaha, NE 68198, United States
Quan P Ly, Department of Surgery, Division of Surgical Oncology, University of Nebraska Medical Center, Omaha, NE 68198, United States
Author contributions: Cathcart SJ read the literature, compiled review data, wrote the first draft of the paper, and repeatedly edited the manuscript; Sasson AR was the treating surgical oncologist and participated in idea and early revisions of the paper; Kozel JA was the treating surgical pathologist and participated in the idea and early revisions of the paper, as well as provided photographs of the gross and microscopic findings; Oliveto JM assisted in reviewing remote and preoperative imaging and provided photographs of CT imaging; Ly QP repeatedly reviewed the manuscript, made surgical recommendations, and approved the final version.
Institutional review board statement: This case report was exempt from the Institutional Review Board standards at the University of Nebraska Medical Center.
Informed consent statement: The patient involved in this study gave his verbal informed consent authorizing use and disclosure of his protected health information.
Conflict-of-interest statement: All authors have no conflicts 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/
Correspondence to: Sahara J Cathcart, MD, Resident, Department of Pathology and Microbiology, 985900 Nebraska Medical Center, Omaha, NE 68198-5900, United States. sahara.cathcart@unmc.edu
Telephone: +1-402-5593456 Fax: +1-402-5596018
Received: December 5, 2016
Peer-review started: December 6, 2016
First decision: January 13, 2017
Revised: February 14, 2017
Accepted: April 23, 2017
Article in press: April 24, 2017
Published online: June 16, 2017
Processing time: 191 Days and 7.8 Hours
Abstract

Gangliocytic paraganglioma (GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindle-like/sustentacular cells, and ganglion-like cells. While this tumor most often behaves in a benign manner, cases with metastasis are reported. We describe the case of a 62-year-old male with a periampullary GP with metastases to two regional lymph nodes who was successfully treated with pancreaticoduodenectomy. Using PubMed, EMBASE, EBSCOhost MEDLINE and CINAHL, and Google Scholar, we searched the literature for cases of GP with regional lymph node metastasis and evaluated the varying presentations, diagnostic workup, and disease management of identified cases. Thirty-one cases of GP with metastasis were compiled (30 with at least lymph node metastases and one with only distant metastasis to bone), with age at diagnosis ranging from 16 to 74 years. Ratio of males to females was 19:12. The most common presenting symptoms were abdominal pain (55%) and gastrointestinal bleeding or sequelae (42%). Twenty-five patients underwent pancreaticoduodenectomy. Five patients were treated with local resection alone. One patient died secondary to metastatic disease, and one died secondary to perioperative decompensation. The remainder did well, with no evidence of disease at follow-up from the most recent procedure (except two in which residual disease was deliberately left behind). Of the 26 cases with sufficient histological description, 16 described a primary tumor that infiltrated deep to the submucosa, and 3 described lymphovascular invasion. Of the specific immunohistochemistry staining patterns studied, synaptophysin (SYN) stained all epithelioid endocrine cells (18/18). Neuron specific enolase (NSE) and SYN stained most ganglion-like cells (7/8 and 13/18 respectively), and S-100 stained all spindle-like/sustentacular cells (21/21). Our literature review of published cases of GP with lymph node metastasis underscores the excellent prognosis of GP regardless of specific treatment modality. We question the necessity of aggressive surgical intervention in select patients, and argue that local resection of the mass and metastasis may be adequate. We also emphasize the importance of pre-surgical assessment with imaging studies, as well as post-surgical follow-up surveillance for disease recurrence.

Keywords: Gangliocytic paraganglioma; Metastases; Duodenum; Lymph node dissection; Pancreaticoduodenectomy

Core tip: Duodenal gangliocytic paragangliomas (GP) generally behave in a benign manner, but infrequently lymph node, and rarely distant, metastases occur. Even in such cases, prognosis remains excellent with only a single reported disease-related death. Here we report a patient with a duodenal GP with regional lymph node metastases and review the literature to help direct management of the rare tumor. In reviewing the literature, achieving complete resection of primary tumor and positive lymph nodes appears to be curative. As such, this should be the therapeutic goal in surgically fit patients.