Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Sep 26, 2021; 9(27): 8071-8081
Published online Sep 26, 2021. doi: 10.12998/wjcc.v9.i27.8071
Primary pancreatic paraganglioma harboring lymph node metastasis: A case report
Cui-Nan Jiang, Xiao Cheng, Jing Shan, Mei Yang, Yu-Qing Xiao
Cui-Nan Jiang, Yu-Qing Xiao, Department of Hepatopancreatobiliary Surgery, Chengdu Third People’s Hospital, Chengdu 610031, Sichuan Province, China
Xiao Cheng, Department of Pathology, Chengdu Third People’s Hospital, Chengdu 610031, Sichuan Province, China
Jing Shan, Mei Yang, Department of Gastroenterology, Chengdu Third People’s Hospital, Chengdu 610031, Sichuan Province, China
Author contributions: Jiang CN collected the relevant clinical data and wrote the manuscript; Cheng X was responsible for the pathological analysis and interpretation; Shan J performed the endoscopic ultrasonography and interpreted the results; Yang M provided processed image data of endoscopic ultrasonography; Xiao YQ and Jiang CN performed the operation; Xiao YQ checked the manuscript and provided suggestions for revision.
Informed consent statement: Informed consent was obtained from the patient for publication of this case report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest to report.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that 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:
Corresponding author: Yu-Qing Xiao, MD, Deputy Director, Surgeon, Department of Hepatopancreatobiliary Surgery, Chengdu Third People’s Hospital, No. 82 Qinglong Street, Qingyang District, Chengdu 610031, Sichuan Province, China.
Received: February 24, 2021
Peer-review started: February 24, 2021
First decision: May 6, 2021
Revised: May 19, 2021
Accepted: August 5, 2021
Article in press: August 5, 2021
Published online: September 26, 2021

Primary pancreatic paragangliomas are extremely rare tumors. Limited by the diagnostic efficacy of histopathological examination, their malignant behavior is thought to be associated with local invasion or metastasis, with only four malignant cases reported in the literature to date. As pancreatic paragangliomas share similar imaging features with other types of pancreatic neuroendocrine neoplasms, they are difficult to diagnose accurately without the support of pathological evidence. As primary pancreatic paragangliomas are rare, especially those accompanied by lymph node metastasis, there is currently no consensus on treatment. Herein, we report a case of primary pancreatic paraganglioma with lymph node metastasis.


A mass located in the pancreatic body was incidentally discovered on computed tomography in a 41-year-old Tibetan man. Distal pancreatectomy was subsequently performed and a 4.1 cm × 4.2 cm tumor was found embedded in the body of the pancreas during surgery. Histological examination confirmed the characteristics of paraganglioma in which the neoplastic chief cells were arranged in a classic Zellballen pattern under hematoxylin-eosin staining. Further, immunohistochemistry demonstrated that the sustentacular cells in the tumor tissue were positive for S-100 protein, and neoplastic cells and pancreatic draining lymph nodes were positive for chromogranin A and synaptophysin; thus, the presence of lymph node metastasis (two of the eight resected pancreatic draining lymph nodes) was also confirmed. A diagnosis of primary pancreatic paraganglioma with lymph node metastasis was finally established. The patient remained disease-free for 1 year after the surgery.


A definite diagnosis of pancreatic paraganglioma mainly depends on postoperative histopathological and immunohistochemical examinations. Surgical resection may be the first treatment of choice for patients with primary pancreatic paraganglioma that has metastasized to the lymph nodes.

Keywords: Paraganglioma, Pancreas, Lymph node metastasis, Distal pancreatectomy, Case report

Core Tip: Paragangliomas are rare neuroendocrine tumors that originate from autonomic nervous systems, with a morbidity of only 2 to 8 in 1000000 of the population. Paragangliomas arising from the pancreas are even less, local invasion or metastasis are thought to be associated with malignant behaviors, and only four of these cases have been reported to date. It is difficult to make an accurate preoperative diagnosis of pancreatic paraganglioma especially when the tumor is non-functioning. No standard therapeutic consensus has been reached yet. Herein, we present a case of primary pancreatic paraganglioma with lymph node metastasis and discuss the related issues.