Surgical management and outcomes of spinal clear cell sarcoma: A retrospective study of five cases and literature review

Abstract Purpose Clear cell sarcoma (CCS) is a rare high-grade malignant tumor accounting for less than 1% of all soft tissue sarcomas. CCS in the spine is much rarer and poorly understood. The objective of our study is to discuss clinical characteristics, surgical management and outcomes of spinal CCS. Methods Between October 2011 and December 2014, five patients with spinal CCS underwent surgical treatment in our center. Five cases from literature focusing on the spinal CCS were also reviewed. We retrospectively analyzed clinical data and outcome of all ten patients to present our understanding of spinal CCS. Results Three en bloc and two piecemeal resections were performed successfully. The syndromes of all the patients were obviously relieved after operation. The mean follow-up period was 24.4 months (range 10–41 months). Two patients died of lung metastasis 10 and 26 months after operation respectively. One patient was alive with tumor recurrence. Other two patients were alive with no evidence of disease at last follow-up. Conclusions Prognosis of spinal CCS tend to be worse than CCS in common sites. En bloc resection could be regarded as the first treatment option. Tumor size, tumor location, preoperative neurological status and resection mode might be the potential prognostic factors of spinal CCS.