Recurrent epithelioid sarcoma in the thoracic spine successfully treated with multilevel total en bloc spondylectomy

Abstract

Epithelioid sarcoma (ES) is a rare type of soft tissue tumor. The common location of ES is at the extremities and rarely occurs in axial skeleton. Only two cases have been reported so far. Initial wide resection is recommended for the treatment of ES. However, the local recurrent rate is high and repeat surgical resection is still an option for the treatment of the recurrent. In the spine, however, the proper treatment of recurrent ES has not yet been published. Therefore, the objective of this case report is to illustrate the management strategies for the local recurrent ES after initial surgical resection in the thoracic spine. A 14-year-old boy was diagnosed for ES in the thoracic spine for 2 years. He was first treated by surgical resection followed by the chemotherapy and radiotherapy but the disease had progressed and the spine was gradually deformed. He was admitted to our facility with a large soft tissue mass, severe kyphotic deformity and neurological deficit. We removed the tumor en bloc by one-stage posterior only approach. The posterior transpedicular spinal instrumentation and fibular strut graft were used for the reconstruction. On the last follow-up, 2 year after the surgery, the patient remained in good condition. In conclusion, the recurrent ES of the spine can still archive a good oncological outcome with repeat radical resection, but the initial radical resection remains the best treatment option in order to retard the relentless course of this kind of malignancy.