Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Sep 26, 2022; 10(27): 9834-9844
Published online Sep 26, 2022. doi: 10.12998/wjcc.v10.i27.9834
Diagnostic features and therapeutic strategies for malignant paraganglioma in a patient: A case report
Lei Gan, Xu-Dong Shen, Yang Ren, Hong-Xia Cui, Zhi-Xiang Zhuang
Lei Gan, Xu-Dong Shen, Yang Ren, Zhi-Xiang Zhuang, Department of Oncology, The Second Affiliated Hospital of Soochow University, Suzhou 215004, Jiangsu Province, China
Hong-Xia Cui, Department of Pathology, The Second Affiliated Hospital of Soochow University, Suzhou 215004, Jiangsu Province, China
Author contributions: Gan L wrote the initial draft of the manuscript; Shen XD, Ren Y, Cui HX, and Zhuang ZX analyzed data, and wrote, edited, and reviewed the manuscript; all authors approved the final version of the manuscript and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Supported by National Natural Sciences Foundation of China, No. 81803553; and National Natural Science Foundation Pre-research Program of China, No. SDFEYGJ1608.
Informed consent statement: The present study was approved by the Ethics Committee of the Second Affiliated Hospital of Soochow University. The patient provided written informed consent form prior to commencing the study.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016) statement, and the manuscript was prepared and revised according to the CARE Checklist (2016) statement.
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 NonCommercial (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: Lei Gan, PhD, Doctor, Department of Oncology, The Second Affiliated Hospital of Soochow University, No. 1055 Sanxiang Road, Gusu District, Suzhou 215004, Jiangsu Province, China.
Received: April 25, 2022
Peer-review started: April 25, 2022
First decision: June 8, 2022
Revised: July 1, 2022
Accepted: August 15, 2022
Article in press: August 15, 2022
Published online: September 26, 2022

Paragangliomas and extra-adrenal pheochromocytomas are uncommon neuroendocrine tumors with ubiquitous distribution. Malignant paraganglioma is a relatively rare entity. We report the treatment and pathological characteristics of a patient with malignant paraganglioma, and summarize the latest advances in the treatment of malignant paraganglioma based on a literature review.


A 45-year-old Chinese woman presented to the hospital due to pain in the waist (right side) and right buttock, and was diagnosed as malignant paraganglioma after the placement of ureteral stent, implantation of ileus catheter, and transvaginal removal of the vaginal mass. After relief of intestinal obstruction, the patient received intravenous chemotherapy and peritoneal perfusion chemotherapy. Although her pelvic mass disease was stable, she developed multiple liver metastases and bone metastases. Due to the development of spinal cord compression, she underwent orthopedic surgery, followed by radiotherapy, and molecular targeted therapy with apatinib, but with poor disease control.


Clinical management of paraganglioma is challenging for endocrinologists and oncologists. Prospective studies are required to develop standardized therapeutic strategies for malignant paragangliomas.

Keywords: Malignant paraganglioma, Chemotherapy, Radiotherapy, Targeted therapy, Case report

Core Tip: Paragangliomas are widely distributed and have diverse clinical manifestations. Although most paragangliomas are benign, the malignancy is involved in approximately 10% of paragangliomas. The traditional treatment of malignant paragangliomas is surgery to the primary site. Surgery followed by adjuvant radiation is used less frequently, and chemotherapy is typically reserved for the distant disease. This study indicated the diagnostic features and therapeutic strategies of malignant paraganglioma. Therapeutic strategies for malignant paragangliomas are lacking. After the initial treatment, the patient’s progression-free survival reached 21 mo. Subsequently, the patient progressed and was treated again with chemoradiotherapy, surgery, and targeted therapy.