Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 26, 2020; 8(22): 5707-5714
Published online Nov 26, 2020. doi: 10.12998/wjcc.v8.i22.5707
Nonhypertensive male with multiple paragangliomas of the heart and neck: A case report
Qi Wang, Zhe-Yong Huang, Jun-Bo Ge, Xian-Hong Shu
Qi Wang, Zhe-Yong Huang, Jun-Bo Ge, Xian-Hong Shu, Department of Cardiology, Zhongshan Hospital, Fudan University, Shanghai Institute of Cardiovascular Diseases, Shanghai 200032, China
Author contributions: Shu XH and Huang ZY advised on this case report; Wang Q wrote the manuscript; Shu XH, Huang ZY, and Ge JB reviewed the manuscript; All authors have read and approve the final manuscript.
Supported by the Youth Fund Program in Zhongshan Hospital, Fudan University, No. 2018ZSQH49.
Informed consent statement: The patient provided written informed consent to the publication of this case report.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
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 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: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Xian-Hong Shu, MD, Vice Director, Department of Cardiology, Zhongshan Hospital, Fudan University, Shanghai Institute of Cardiovascular Diseases, No. 180 Fenglin Road, Xuhui District, Shanghai 200032, China. shu.xianhong@zs-hospital.sh.cn
Received: July 24, 2020
Peer-review started: July 24, 2020
First decision: September 12, 2020
Revised: September 22, 2020
Accepted: October 20, 2020
Article in press: October 20, 2020
Published online: November 26, 2020
Processing time: 124 Days and 3.2 Hours
Abstract
BACKGROUND

Paraganglioma is a rare disease that can be lethal if undiagnosed. Thus, quick recognition is very important. Cardiac paragangliomas are found in patients who have hypertension. The classic symptoms are the triad of headaches, palpitations, and profuse sweating. We describe a very rare case of multiple paragangliomas of the heart and bilateral carotid artery without hypertension and outline the management strategies for this disease.

CASE SUMMARY

A 46-year-old man presented with the chief complaint of recently recurrent chest pain with a history of hemangioma of the bilateral carotid artery that had been surgically removed. He was found to have an intracardiac mass in the right atrioventricular groove and underwent successful excision. The final pathology demonstrated that the intracardiac mass was a cardiac paraganglioma, and the patient had an increased level of normetanephrine in the blood. The pathology and immunohistochemistry results showed that the bilateral carotid masses were also paragangliomas. During the 3 mo follow-up period, the patient did not experience recurrence of chest pain.

CONCLUSION

To our knowledge, this is the first case of multiple paragangliomas of the heart and neck without hypertension. This rare disease can be lethal if left undiagnosed. Thus, quick recognition is very important. The key to the diagnosis of cardiac paraganglioma is the presence of typical symptoms, including headaches, palpitations, profuse sweating, hypertension, and chest pain. Radiology can demonstrate the intracardiac mass. It is important to determine the levels of normetanephrine in the blood. The detection of genetic mutations is also recommended. Surgical resection is necessary to treat the disease and obtain pathological evidence.

Keywords: Chest pain; Intracardiac mass; Cardiac paraganglioma; Normetanephrine; Pathological evidence; Case report

Core Tip: Paraganglioma is a very rare disease. The clinical presentation of paraganglioma is dependent on the symptoms caused by local invasion and/or hypersecretion of catecholamines. The classic symptoms are the triad of headaches, palpitations, and profuse sweating. Surgical resection is necessary to treat the disease and obtain pathological evidence. We describe a first case of multiple paragangliomas of the heart and neck without hypertension and management with surgical resection.