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.

Multilevel En Bloc Spondylectomy for Tumors of the Thoracic and Lumbar Spine Is Challenging But Rewarding

“…Thirty-one patients (81%) were alive without any evidence of disease. Other case series and case reports in the literature have also shown total en bloc spondylectomy to be effective in increasing patient survival and preventing local recurrences [1,3,5,6,8,9,13,14,20] (Table 2). Boriani et al [1], in their study of 33 patients undergoing multilevel en bloc spondylectomy, had a disease-free survival rate of more than 50%.…”

“…All living patients recovered from their complications at final followup. In the literature, the majority of patients treated with multilevel en bloc spondylectomy recover from their complications [3,6,8,9,14,20]. Multilevel en bloc resections are technically more demanding and morbid, owing to the proximity of neurovascular structures, visceral organs, need for an anterior and posterior approach, and increased blood loss and surgical time, thus increasing the risk of complications.…”

“…1, 2; supplemental materials are available with the online version of CORR 1 ), four levels in three patients (Figs. [3][4][5], and five levels in one patient (Suppl Fig. 3; supplemental materials are available with the online version of CORR 1 ).…”

“…It becomes further challenging when the tumor involves multiple levels and visceral structures. There are a few reports in the literature on multilevel en bloc procedures, although most are case reports and small series [1,3,5,6,8,9,13,14,20].…”

“…(2) Is reconstruction after this procedure feasible? (3) What complications are associated with this procedure? (4) is it possible to achieve adequate surgical margins with this procedure?…”