Treatment and outcomes of epithelioid sarcoma of the spine

Epithelioid sarcoma (ES) is a rare soft-tissue neoplasm which is most commonly found in the extremities of young adult males. ES has a poor prognosis due to its aggressiveness as it frequently recurs locally and can undergo lymphatic metastasis to soft tissue, fascia, bone, lymph nodes, lung, and brain. The most common form is the classic-type (granuloma-like), though a more aggressive subtype known as the proximal- or axial-type has also been described. As ES of the spine is exceedingly rare, with only seven patients being reported in the literature, the outcomes of these patients is unclear. We have reviewed the literature of all existing spinal ES cases to recommend treatment strategies and report the first case of proximal-type ES in the cervical spine. Patients with spinal ES had an average age of 20.7 years, with 71.4% of cases being in males. Metastasis was common and was found in 83.3% of patients, with lung metastasis being found in 60% of these patients. Due to the high rates of local recurrence and distant metastasis, the goal of surgery remains gross total resection of all tumor and involved bony elements if feasible without significant neurological deficits. Ligation of involved nerve roots may be necessary to achieve adequate resection of the tumor mass as nerve sheaths can serve as a pathway for extension. In the cervical spine, resection of these lesions is difficult due to involvement of the vertebral arteries in addition to nerve roots, increasing the surgical risk.