Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jan 6, 2019; 7(1): 109-115
Published online Jan 6, 2019. doi: 10.12998/wjcc.v7.i1.109
Bilateral and symmetric C1-C2 dumbbell ganglioneuromas associated with neurofibromatosis type 1: A case report
Chun-Yu Tan, Jia-Wei Liu, Yi Lin, Xin-Xin Tie, Peng Cheng, Xue Qi, Yue Gao, Zong-Ze Guo
Chun-Yu Tan, Jia-Wei Liu, Yi Lin, Xin-Xin Tie, Peng Cheng, Zong-Ze Guo, Department of Neurosurgery, The First Hospital of China Medical University, Shenyang 110000, Liaoning Province, China
Xue Qi, X-ray Department, The First Hospital of China Medical University, Shenyang 110000, Liaoning Province, China
Yue Gao, Department of Pathology, The First Hospital of China Medical University, Shenyang 110000, Liaoning Province, China
Author contributions: Tan CY and Liu JW contributed equally to this work; Tan CY and Liu JW designed the report; Lin Y, Tie XX, Cheng P, Qi X, Gao Y, and Guo ZZ collected the clinical data of the patient; Tan CY and Liu JW analyzed the data and wrote the paper.
Informed consent statement: The patient involved in this study gave his written informed consent authorizing use and disclosure of his protected health information.
Conflict-of-interest statement: All the authors have no conflicts of interests to declare.
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 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: Zong-Ze Guo, MD, PhD, Doctor, Professor, Department of Neurosurgery, The First Hospital of China Medical University, 155 Nanjingbei Street, Heping, Shenyang 110000, Liaoning Province, China. cmuguozongze@hotmail.com
Telephone: +86-24-83283133 Fax: +86-24-83283706
Received: September 4, 2018
Peer-review started: September 4, 2018
First decision: October 12, 2018
Revised: October 25, 2018
Accepted: December 7, 2018
Article in press: December 8, 2018
Published online: January 6, 2019
Abstract
BACKGROUND

Ganglioneuroma (GN) is a rare and benign tumor that originates from autonomic nervous system ganglion cells. The most frequently involved sites are the posterior mediastinum, the abdominal cavity, and the retroperitoneal space. It rarely occurs in the cervical area, compressing the spinal cord. Neurofibromatosis type 1 (NF-1) is an autosomal dominant inheritance disorder, whose prevalence rate approximates one per 3000.

CASE SUMMARY

We report an extremely rare case of bilateral and symmetric dumbbell GNs of the cervical spine with NF-1. A 27-year-old man with NF-1 presented with a one-year history of gradually progressive right upper extremity weakness and numbness in both hands. Magnetic resonance imaging showed bilateral and symmetric dumbbell lesions at the C1-C2 levels compressing the spinal cord. We performed total resection of bilateral tumors, and the postoperative histopathological diagnosis of the resected mass was GN. After operation, the preoperative symptoms were gradually relieved without complications. To our knowledge, this is the sixth report of cervical bilateral dumbbell GNs.

CONCLUSION

In some cases, cervical bilateral dumbbell GNs could be associated with NF-1. The exact diagnosis cannot be obtained before operation, and pathological outcome is the current gold standard. Surgical resection is the most effective option, and disease outcome is generally good after treatment.

Keywords: Ganglioneuroma, Cervical, Bilateral, Dumbbell, Surgery, Case report

Core tip: Ganglioneuroma is the final mature form of neuroblastic tumors which mainly involves the posterior mediastinum and the retroperitoneal space. We report a rare case of cervical bilateral dumbbell ganglioneuromas associated with neurofibromatosis type 1. Only five cases have been reported. Due to non-specific symptoms, signs, and radiological images, it is difficult to make a definite diagnosis before postoperative pathology. The prognosis of this tumor is good. Complete resection is the exhaustive treatment. Furthermore, incomplete resection does not increase the risk of progression if tumor residuals are smaller than 2 cm.