Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. May 6, 2022; 10(13): 4301-4313
Published online May 6, 2022. doi: 10.12998/wjcc.v10.i13.4301
Primary intracranial extraskeletal myxoid chondrosarcoma: A case report and review of literature
Zi-You Zhu, Yu-Bo Wang, Han-Yi Li, Xin-Min Wu
Zi-You Zhu, Yu-Bo Wang, Xin-Min Wu, Department of Neurosurgery, The First Hospital Affiliated to Jilin University, Changchun 130021, Jilin Province, China
Han-Yi Li, Department of Orthodontics, Hospital of Stomatology of Jilin University, Changchun 130021, Jilin Province, China
Author contributions: Zhu ZY, Wang YB and Li HY contributed equally to this work; Wu XM, Wang YB were the surgeons that performed the surgery; Wu XM designed the study, Zhu ZY and Wang YB made data collection, data analysis and interpretation, and made contribution to interpreting of the imaging findings, reviewing the literatures, and writing the manuscript; Li HY and Zhu ZY contributed to the translation work, made terms and grammar corrections; Wu XM, Zhu ZY and Wang YB contributed to the revision work; All authors contributed to the article and issued the final approval for the version to be submitted.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict 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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Xin-Min Wu, MD, PhD, Professor, Department of Neurosurgery, The First Hospital Affiliated to Jilin University, No. 1 Xinmin Street, Chaoyang District, Changchun 130021, Jilin Province, China. wuxinmin@jlu.edu.cn
Received: December 29, 2021
Peer-review started: December 29, 2021
First decision: January 25, 2022
Revised: February 4, 2022
Accepted: March 6, 2022
Article in press: March 6, 2022
Published online: May 6, 2022
Processing time: 121 Days and 21.7 Hours
Abstract
BACKGROUND

Primary intracranial extraskeletal myxoid chondrosarcoma (EMC) is an extremely rare low- to intermediate-grade malignant soft tissue sarcoma, and only 15 cases have been reported in the literature. Due to its rarity, clinical data and research on this tumor type are extremely limited, the pathogenesis and histological origin are still unclear, and the diagnostic and standard clinical treatment strategies for intracranial EMC remain controversial and undefined.

CASE SUMMARY

We reported a case of a 52-year-old male who was admitted to the hospital with headache and dizziness for 1 mo, and his health status deteriorated during the last week. CT of the head showed a well-defined low-density lesion situated in the left cavernous sinus. Brain magnetic resonance imaging (MRI) showed a 3.4 cm × 3.0 cm sized, well-defined, round-shaped and heterogeneously enhanced lesion located in the left cavernous sinus. The entire lesion was removed via supratentorial craniotomy and microsurgery. Postoperative pathological diagnosis indicated primary intracranial EMC. Subsequently, the patient underwent 45 Gy/15 F stereotactic radiotherapy after discharge. At present, it is 12 mo after surgery, with regular postoperative follow-up and regular MRI examinations, that there are no clinical symptoms and radiographic evidence indicating the recurrence of the tumor, and the patient has returned to normal life.

CONCLUSION

Currently, the most beneficial treatment for primary intracranial EMC is gross total resection combined with postoperative radiotherapy. Long-term follow-up is also necessary for patients.

Keywords: Extraskeletal myxoid chondrosarcoma; Primary intracranial neoplasm; Soft tissue sarcoma; Surgery; Radiotherapy; Case report

Core Tip: Primary intracranial extraskeletal myxoid chondrosarcoma (EMC) is an extremely rare intracranial neoplasm, and only 15 cases have been reported in the literature. We report herein an extremely rare case, which is also the first case of primary EMC occurring in the cavernous sinus. Primary intracranial EMC is indolent in growth yet has a high recurrence rate after total resection. In these cases, we observed the importance of postoperative radiotherapy that can improve the outcome of patients with primary intracranial EMC. Surgical total resection combined with postoperative radiotherapy can prolong progression-free survival and decrease the recurrence rate. Meanwhile, long-term follow-up is also necessary for patients after surgery. In addition, primary intracranial EMC should also be considered when diagnosing and distinguishing a lesion in the cavernous sinus.