Nan YH, Chiu CD, Chen WL, Chen LC, Chen CC, Cho DY, Guo JH. Epithelioid angiosarcoma of the cervical spine: A case report. World J Clin Cases 2025; 13(17): 101593 [DOI: 10.12998/wjcc.v13.i17.101593]
Corresponding Author of This Article
Jeng-Hung Guo, MD, PhD, Chief Physician, Department of Neurosurgery, China Medical University Hospital, No. 2 Yude Road, Taichung 404327, Taiwan. cmuhd14299@gmail.com
Research Domain of This Article
Surgery
Article-Type of This Article
Case Report
Open-Access Policy of This Article
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/
Yu-Han Nan, Cheng-Di Chiu, Chun-Chung Chen, Der-Yang Cho, Jeng-Hung Guo, Department of Neurosurgery, China Medical University Hospital, Taichung 404327, Taiwan
Cheng-Di Chiu, Der-Yang Cho, Graduate Institute of Biomedical Science, China Medical University, Taichung 404328, Taiwan
Cheng-Di Chiu, Jeng-Hung Guo, Spine Center, China Medical University Hospital, Taichung 404327, Taiwan
Wei-Liang Chen, Department of Interventional and Diagnostic Neuroradiology, China Medical University Hospital, Taichung 404327, Taiwan
Liang-Chi Chen, Department of Pathology, China Medical University Hospital, Taichung 404327, Taiwan
Chun-Chung Chen, Graduate Institute of Integrated Medicine, China Medical University, Taichung 404328, Taiwan
Author contributions: Nan YH performed the literature reviews and wrote the manuscript; Guo JH performed the surgery and planned the research in the manuscript; Chiu CD, Chen WL, Chen LC, Chen CC and Cho DY provided refinement suggestions for this article and verified the correctness of the manuscript.
Informed consent statement: The study participant's legal guardian provided informed written consent prior to study enrollment.
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), 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: Jeng-Hung Guo, MD, PhD, Chief Physician, Department of Neurosurgery, China Medical University Hospital, No. 2 Yude Road, Taichung 404327, Taiwan. cmuhd14299@gmail.com
Received: September 20, 2024 Revised: December 10, 2024 Accepted: January 23, 2025 Published online: June 16, 2025 Processing time: 151 Days and 4.9 Hours
Abstract
BACKGROUND
Epithelioid angiosarcoma (EA) is an aggressive, malignant endothelial-cell tumor of vascular or lymphatic origin. EA often arises from deep soft tissues such as pleura, breast, bone and gastrointestinal tract. It usually affects patients aged 60-70 years and is associated with high recurrence and metastasis rates with surgical resection as the primary treatment of choice. Overall survivals are generally poor, ranging from 6 to 16 months. More than 50% of patients died of disease within 2 to 3 years of diagnosis.
CASE SUMMARY
We present a rare case of EA of the cervical spine causing a C6 pathological fracture complicated by severe kyphosis. The patient received C4-7 posterior laminectomy and C2/3/4/7/T1 transpedicular screw fixation, followed by anterior C5-6 corpectomy with allograft bone fusion and cervical plate fixation. Postoperative radiotherapy was administered without delay. However, the patient died of rapidly progressive acute respiratory distress syndrome 3 weeks after the second surgery.
CONCLUSION
EA with spinal involvement is extremely rare. Early detection and cord decompression may prevent neurological deterioration and preserve better quality of life.
Core Tip: Epithelioid angiosarcoma (EA) is an aggressive and rare malignant tumor, particularly with spinal involvement. This case report presents a case of cervical spinal EA in a 63-year-old male with a pathological fracture causing spinal cord compression. The patient underwent staged surgeries with decompressive laminectomy, corpectomy, and bone fusion, followed by radiotherapy. Despite aggressive management, the patient succumbed to acute respiratory distress syndrome. This case emphasizes the importance of early diagnosis, timely tumor decompression to prevent neurological deterioration, and the challenges in managing EA due to its high recurrence and metastasis rates. Further research is needed to improve therapeutic outcomes.