Published online Jun 16, 2025. doi: 10.12998/wjcc.v13.i17.101593
Revised: December 10, 2024
Accepted: January 23, 2025
Published online: June 16, 2025
Processing time: 151 Days and 4.9 Hours
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.
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.
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.